PREP 2022

Question 1

Absent pulmonary valve syndrome is most commonly associated with what?

Answer 1

ToF

Question 2

Absent pulmonary valve syndrome occurs in what % of ToF?

Answer 2

2.5%

Question 3

True or False: In absent pulmonary valve syndrome, there can be complete absence of pulmonary valve tissue or a small ridge of tissue at the valve annulus which is often hypoplastic?

Answer 3

True

Question 4

What is the presentation of absent pulmonary valve syndrome?

Answer 4

Varies widely from minimal symptoms to respiratory distress/failure

Question 5

What causes respiratory distress in absent pulmonary valve syndrome?

Answer 5

Proximal and/or distal bronchial compression

Question 6

What can be done to improve respiratory distress in absent pulmonary valve syndrome?

Answer 6

-Prone position (reduces bronchial compression by the PAs and decreases ventilation/perfusion mismatch)
-Intubation/PPV for patients with persistent respiratory distress

Question 7

How can ToF absent PV syndrome result in cyanosis?

Answer 7

R-L shunting at the VSD

Question 8

True or False: Patients with ToF absent PV syndrome are dependent on unrestrictive mixing at the atrial level?

Answer 8

False

Question 9

The absence of what is hypothesized to be associated with the development of absent pulmonary valve syndrome?

Answer 9

PDA (only rare cases where absent pulmonary valve syndrome develops with the presence of a PDA)

Question 10

Respiratory distress in ToF absent PV syndrome is often the result of what?

Answer 10

Dilated pulmonary arteries causing bronchial compression

Question 11

What can help to decrease ventilation/perfusion mismatch in ToF absent PV syndrome?

Answer 11

Prone positioning (moves that PAs off the airway to decrease bronchial compression)

*Patients with persistent respiratory distress may require intubation and PPV

Question 12

How is the RP relationship determined?

Answer 12

-Draw a line at the midway point between 2 R waves
-If the P wave falls in the first half of the RR interval, it is short RP tachycardia
-If the P wave falls in the second half of the RR interval, it is long RP tachycardia

Question 13

What is the differential diagnosis for a long RP tachycardia?

Answer 13

-Atrial ectopic tachycardia
-PJRT
-Atypical AVNRT
-Sinus tachycardia

Question 14

Describe the P-waves in PJRT

Answer 14

Deeply negative in the inferior leads (II, III, aVF)

Question 15

Describe the P-waves in sinus tachycardia?

Answer 15

-Normal P-wave axis and morphology (positive in I and aVF)

Question 16

Long RP tachycardia with low-amplitude P waves and a biphasic P-wave in lead I… most likely etiology?

Answer 16

EAT- Long RP tachycardia with abnormal P-waves, but not PJRT (P-waves would be negative in II, III, aVF)

Question 17

True or False: Patients with EAT can have incessant tachycardia?

Answer 17

True- Rate may not be significantly elevated and patients can be asymptomatic delaying diagnosis…. longer duration of tachycardia before diagnosis, greater risk of patient developing tachycardia induced cardiomyopathy

Question 18

What should be done before treating EAT?

Answer 18

Echo- need to assess for structural abnormalities and ventricular dysfunction

Question 19

What is the typical first line therapy for EAT if function is preserved?

Answer 19

B-blockers

Question 20

After beta-blockers, what are the next medications used for EAT?

Answer 20

-Sotalol or Flecainide
-Amiodarone as a 3rd line agent

Question 21

Besides antiarrhythmics, what can be done to treat EAT?

Answer 21

Ablation- Potentially curative with high success rates and low complication rates

*Should be considered 1st line if patient is adequate size (and if there is ventricular dysfunction)

Question 22

Describe the RP relationship in SVT due to WPW and typical AVNRT

Answer 22

Short RP

Question 23

What is the differential diagnosis for long RP tachycardia?

Answer 23

-EAT
-PJRT
-Atypical AVNRT
-Sinus tachycardia

Question 24

Patients with incessant atrial tachycardia are at risk of developing what?

Answer 24

Tachycardia induced cardiomyopathy (more common when HR isn’t significantly elevated)

Question 25

Failure to thrive, macroglossia, mild respiratory distress, cardiomegaly, hepatomegaly, mild generalized hypotonia, elevated CK and transaminases?

Answer 25

Classic infantile-onset Pompe

Question 26

In classic infantile-onset Pompe disease, enzyme assay will shoe a severe deficiency of what?

Answer 26

Acid alpha glucosidase

Question 27

What is a lysosomal storage disorder characterized by deficiency or complete absence of the lysosomal enzyme, acid alpha glucosidase (GAA)?

Answer 27

Pompe -Also known as glycogen storage disease type II or acid maltase deficiency

Question 28

What causes Pompe disease?

Answer 28

Autosomal recessive mutation of the GAA gene on chromosome 17q25

Question 29

What does deficiency of acid alpha glucosidase result in?

Answer 29

Accumulation of glycogen in lysosomes and cytosol of target organs (skeletal muscle, cardiac muscle, liver)

Question 30

How does Pompe disease present?

Answer 30

Wide spectrum of disease manifestations that vary in time of clinical onset, severity of symptoms, rapidity of disease progression and overall prognosis

Question 31

What does the disease severity in Pompe correlate with?

Answer 31

Amount of residual GAA (alpha glucosidase) activity

Question 32

How is Pompe disease calssified?

Answer 32

-Infantile onset -Late onset *According to age of initial clinical presentation

Question 33

When and how does classic infantile Pompe disease usually present?

Answer 33

-1st 2 months of life -Generalized hypotonia, cardiomegaly, respiratory distress, feeding difficulties, failure to thrive

Question 34

What is the characteristic cardiac finding in Pompe patietns?

Answer 34

HCM

Question 35

What causes HCM in Pompe patietns?

Answer 35

Deposition of glycogen into the myocardium

Question 36

Besides HCM, what other cardiac findings can be seen in Pompe disease?

Answer 36

LVNC

Question 37

What is often seen on echo in Pompe patients?

Answer 37

LVOTO due to HCM

Question 38

What is seen on ECG in Pompe disease?

Answer 38

-Short PR interval -Tall QRS complexes with increased voltages -Increased QT dispersion

Question 39

What is often seen in a later stage of infantile Pompe disease?

Answer 39

Progressive dilation of the ventricle

Question 40

What is the natural history of Pompe disease if left untreated?

Answer 40

Death by 1-2 years due to cardiorespiratory failure

Question 41

Patients with classic infantile Pompe disease that what % of residual GAA (alpha glucosidase) activity?

Answer 41

<1%

Question 42

How does non-classic infantile Pompe disease present?

Answer 42

1st year after birth, but is associated with less severe cardiomyopathy and slower disease progression

Question 43

How does late-onset Pompe disease present?

Answer 43

-Any time after infancy as a progressive skeletal myopathy -Typically not associated with any cardiac manifestations -Enzyme assay with 4-40% of residual GAA (alpha glucosidase) activity

Question 44

What is the specific treatment for Pompe disease?

Answer 44

-Enzyme replacement therapy using recombinant human GAA (alpha glucosidase)... has been available since 2006

Question 45

True or False: In infantile Pompe disease, early institution of enzyme replacement therapy has been shown to decrease LV mass, improve skeletal muscle function and prolong overall survival?

Answer 45

True

Question 46

True or False: In infantile Pompe disease, early institution of enzyme replacement therapy improves LV voltages, QT dispersion and PR intervals on ECG?

Answer 46

True

Question 47

True or False: In late-onset disease, enzyme replacement therapy has been shown to slow the progression of muscle weakness?

Answer 47

True

Question 48

True or False: Pompe disease is on the newborn screening protocol in many states?

Answer 48

True (since 2015)

Question 49

When is the best time to initiate enzyme replacement therapy in infantile Pompe disease?

Answer 49

First few days after birth

Question 50

Severe aortic root dilation is commonly seen in what?

Answer 50

Connective tissue disorders like Marfan

Question 51

True or False: It would be quite unusual to see a dilated LV with severely decreased LV function in the early phase of infantile Pompe?

Answer 51

True- can be seen at a later stage in classic infantile Pompe disease when the ventricular function deteriorates and the heart dilates

Question 52

True or False: Pericardial effusion is not specifically associated with infantile Pompe disease?

Answer 52

True

Question 53

What is a glycogen storage disorder caused by a deficiency or complete absence of lysosomal acid alpha glucosidase?

Answer 53

Pompe

Question 54

How does classic infantile Pompe disease present?

Answer 54

Rapidly progressive entity manifesting in early infancy with hypotonia, HCM and respiratory distress

Question 55

What is essential for survival beyond infancy in classic infantile Pompe disease?

Answer 55

Prompt diagnosis and initiation of enzyme replacement therapy with recombinant human acid alpha glucosidase

Question 56

True or False: Late-onset Pompe disease isn't typically associated with cardiac manifestations?

Answer 56

True

Question 57

What is normal velocity flow in the ductus arteriosus on a fetal echocardiogram?

Answer 57

0.6-1.4m/sec

Question 58

What is the size of the ductus arteriosus equivalent to in fetal life?

Answer 58

Pulmonary trunk and descending aorta

Question 59

True or False: In fetal life, the ductus arteriosus carries the majority of the fetal RV output into the descending aorta?

Answer 59

True

Question 60

Depending on gestational age, what % of the RV output actually flows into the developing lungs?

Answer 60

10-25%

Question 61

How is ductal patency maintained during fetal life?

Answer 61

Regulatory action of prostaglandins, nitric oxide, and low fetal O2 saturations

Question 62

What causes premature ductal constriction in a fetus?

Answer 62

-Extrinsic agents: NSAIDS like indomethacin or herbal supplements -Idiopathic *Uncommon and often underrecognized, but can have bad consequences

Question 63

What is the result of prenatal ductal constriction and ductal closure?

Answer 63

-Acute increase in fetal RV afterload -Results in RVH, TR, RAE and increase R-L shunting across the foramen (depends on severity of ductal constriction) -Increased pressure in pulmoanry vessels can cause dilation of MPA and branches with PS, PI or pulmonary vascular disease (thickening of media of pulmonary arterioles in response to obligatory increased flow to lung parenchyma) -Severe cases- progressive RV failure resulting in hydrops fetalis and fetal demise

Question 64

What is the most common echo finding in premature ductal constriction?

Answer 64

Disproportionate RVH

Question 65

What are some findings on fetal echo in premature ductal constriction?

Answer 65

-Disproportionate RVH (most common) -Ductus arteriosus narrow (much smaller than adjacent pulmonary trunk) -Color Doppler with high-velocity jet across ductus arteriosus into descending aorta -PI <1.9 -RV dilation -TR -Right heart failure

Question 66

What is the pulsatility index?

Answer 66

-Measure of resistance to flow -Peak systolic velocity-End diastolic velocity/Mean velocity

Question 67

What is the normal range of pulsatility index for the ductus arteriosus?

Answer 67

1.9-2.5

Question 68

In the presence of ductal constriction, what is the pulsatility index?

Answer 68

<1.9

Question 69

What findings on fetal echo predict imminent fetal hydrops?

Answer 69

-Prominent reversal in ductus venosus flow with atrial contraction -Presence of umbilical venous pulsations

Question 70

What should be done if ductal constriction is seen on fetal echo?

Answer 70

-Close serial follow-up -Maternal history to assess for possible causes (NDAIDS, etc) and discontinue is able -Consider delivery with worsening RV function and impending hydrops (after birth PVR drops and decreases RV afterload)

Question 71

What do the postnatal manifestations and overall outcome of ductal constriction in utero depend on?

Answer 71

Severity, duration and gestational age at onset

Question 72

True or False: Ductal constriction is rare prior to 27 weeks gestation and the risk increases with advancing gestational age?

Answer 72

True

Question 73

What are postnatal symptoms of ductal constriction?

Answer 73

-Mild cyanosis -PPHN -Severe respiratory distress -Neonatal death

Question 74

What may be needed postnatally for an infant with ductal construction?

Answer 74

-Close monitoring -O2 -Pulmonary vasodilators -Ventilator support -ECMO

Question 75

True or False: In some instances, fetal ductal closure is diagnosed postnatally in a cyanotic neonate with disproportionately thickened RV, R-L shunt at atrial level and absent ductus arteriosus within 24 hours of birth?

Answer 75

True

Question 76

What is the result of premature constriction and closure of the fetal ductus arteriosus?

Answer 76

Acute increase in the RV afterload in the fetus *Severe cases, progressive RV failure resulting in hydrops and fetal demise

Question 77

What are the key echo findings in premature ductal cosntriction?

Answer 77

Disproportionate RVH, narrow ductus arteriosus with accelerated flow, ductal PI of <1.9

Question 78

What can occur postnatally due to premature ductal construction?

Answer 78

-Mild cyanosis -PPHN -Severe respiratory distress -Neonatal death *Depends on the degree, duration and developmental stage at onset of ductal constriction

Question 79

What are the major Jones criteria for acute rheumatic fever in the low-risk population?

Answer 79

-Carditis: Clinical or subclinical -Polyarthritis -Syndenham chorea -Erythema marginatum -Subcutaneous nodules

Question 80

What are the minor Jones criteria for acute rheumatic fever in the low-risk population?

Answer 80

-Polyarthralgia -Fever (>38.5C) -ESR >60 and/or CRP >3 -Prolonged PR interval (unless carditis is a major criterion)

Question 81

What is required to diagnose acute rheumatic fever?

Answer 81

2 major 1 major + 2 minor Evidence of preceding strep infection

Question 82

Rheumatic carditis is seen in what % of acute rheumatic fever cases?

Answer 82

40-70%

Question 83

Where is the inflammation in rheumatic carditis?

Answer 83

All layers of the heart (pancarditis)

Question 84

What is the predominant feature in rheumatic carditis?

Answer 84

Valvulitis

Question 85

What is the most common cardiac manifestation of acute rheumatic carditis?

Answer 85

-MR (occurs in 95% of cases) -Then AI (20% with MR and 5% isolated AI)

Question 86

What is the clinical presentation of rheumatic carditis?

Answer 86

Ranges from asymptomatic murmur to acute severe heart failure

Question 87

What is often an early sign of carditis?

Answer 87

Tachycardia

Question 88

Describe the murmur of MR that can be seen in carditis due to acute rheumatic fever?

Answer 88

-High-pitched -Holosystolic -Cardiac apex with radiation to the axilla -Often soft (even if significant regurgitation) -May hear a middiastolic rumbling murmur of relative MS (Carey-Coombs murmur)... but isolated MS doesn't occur in acute rheumatic carditis

Question 89

True or False: In carditis due to acute rheumatic fever, mild MR often resolves whereas moderate to severe MR often progresses to chronic rheumatic heart disease?

Answer 89

True

Question 90

What is the most common valvular lesion in adults with chronic rheumatic heart disease?

Answer 90

Mitral stenosis *Can initially have MR that improves with subsequent development of MS many years later *AS is another late sequelae of rheumatic fever, occurring 20-30 years after the acute episode

Question 91

Describe the murmur heard with isolated AI

Answer 91

-Soft -Early diastolic -Decrescendo *Isolated AI can occur with acute rheumatic carditis, but isn't the most common manifestation

Question 92

How is rheumatic carditis classified?

Answer 92

Pancarditis, but is predominantly a valvulitis

Question 93

What is the most common valvular abnormality in acute rheumatic fever?

Answer 93

MR

Question 94

What is the natural history with mild MR in acute rheumatic fever?

Answer 94

Often resolves

Question 95

What is the natural history with moderate-severe MR in rheumatic heart disease?

Answer 95

Often progresses to chronic rheumatic heart disease

Question 96

Describe what happens in a double chambered RV?

Answer 96

-Perimembranous VSD and development of obstructive RV muscle bundles -Leads to a higher-pressure RV proximal to the RV muscle bundles and a lower-pressure RV distal to the RV muscle bundles -Proximal portion of RV has a high RV pressure (and high TR jet gradient and low VSD gradient) -There will be a high gradient across the RV muscle bundle -PV is usually normal and there will be no significant additional gradient across the pulmonary valve -PA pressure is normal

Question 97

When does double-chambered RV usually present?

Answer 97

-Infancy or childhood -Can be diagnosed in adults

Question 98

Double-chambered RV is frequently associated with what other form of congenital heart disease?

Answer 98

Perimembranous VSD (90% of cases) *VSD may not be seen in a patient with isolated double chambered RV, but there may have been a VSD that closed spontaneously

Question 99

What is often seen in association with a VSD and double-chambered RV?

Answer 99

Subaortic membrane

Question 100

What other forms of CHD is double chambered RV associated with?

Answer 100

-Perimembranous VSD -Subaortic membrane -ToF -Ebstein -DORV -Single ventricle variants -Other forms of CHD

Question 101

Describe the development of double chambered RV from a pathological perspective

Answer 101

-Progressive hypertrophy of anomalous RV muscle located below (proximal to) the infundibular septum -Includes hypertrophy of the crista supraventricularis, trabecular tissue, or moderator band

Question 102

What causes double chambered RV?

Answer 102

-Abnormal blood flow patterns within RV may lead to abnormal RV muscular hypertrophy -Often develops as a perimembranous VSD decreases in size- possibly due to higher flow gradient across the VSD entering the RV body and anomalous RV muscle bundles -Also may be that VSD becomes smaller or closes spontaneously as adjacent anomalous RV muscle bundles hypertrophy with increasing gradient/obstruction from the double chambered RV -Can also develop in patients who have undergone surgery for VSD closure

Question 103

What do the symptoms associated with double chambered RV depend upon?

Answer 103

Degree of obstruction and presence of associated congenital heart abnormalities

Question 104

What are some of the possible symptoms associated with double chambered RV?

Answer 104

-Exercise intolerance -Dyspnea -Syncope -Chest pain -Arrhythmias -Combination *Most children are asymptomatic

Question 105

What has been reported in patients with unrepaired double chambered RV that can lead to acute symptoms caused by a sudden increase in the obstruction?

Answer 105

Endocarditis

Question 106

What are some of the physical exam findings seen in double chambered RV?

Answer 106

-SEM without a valve click (differentiates from RV obstruction from valvar PS) -RV heave or thrill (if moderate to high gradient and if RV proximal to level of obstruction is significantly hypertrophied)

Question 107

What is seen on ECG in double chambered RV?

Answer 107

RV hypertrophy

Question 108

How is double chambered RV diagnosed?

Answer 108

-Children: Echo is usually sufficient (subcoastal or parasternal RV inflow-outflow imaging -Adults: May need TEE, CT, MRI, cath or combination

Question 109

What is done for surgical repair of double chambered RV?

Answer 109

-Resection of abnormal RV muscle, usually through the TV -Presence of any associated cardiac abnormalities (VSD, etc. should be evaluated and addressed at the same time)

Question 110

When is surgical repair of double chambered RV indicated?

Answer 110

-Peak pressure >40mmHg within RV body -Increasing gradient -Evidence of cardiac-related symptoms

Question 111

True or False: Surgical repair of uncomplicated double chambered RV is highly successful with low morbidity and morality?

Answer 111

True- Reoperation for recurrent double chambered RV is unusual when there has been adequate surgical relief of RV obstruction

Question 112

True or False: Transcatheter intervention via balloon angioplasty or stent placement is not usually successful as definitive repair of double chambered RV, but it has been performed in patients who are at high surgical risk?

Answer 112

True

Question 113

Double chambered RV is most commonly seen in what patients?

Answer 113

Young patients in association with a restrictive perimembrnaous VSD

Question 114

True or False: Most children with double chambered RV are asymptomatic?

Answer 114

True

Question 115

When is surgical repair via resection of abnormal RV muscle bundles indicated for double chambered RV?

Answer 115

-Gradient greater than 40mmHg across the anomalous RV muscle bundles -Presence of symptoms -Associated anomalies that warrant surgical intervention

Question 116

What is important as part of screening for adults with repaired CoA?

Answer 116

3D imaging

Question 117

How often should an adult with repaired CoA have 3D imaging?

Answer 117

Every 5 years or less (depending on complexity of case)

Question 118

Besides re-coarctation, what is a risk in patients who have undergone repair of coarctation?

Answer 118

Aneurysm at site of prior repair

Question 119

Which patients with repaired CoA and are at increased risk for aneurysm at site fo repair?

Answer 119

Those who have undergone a polyethylene terephthalate (Dacron) graft repair

Question 120

What is often required to diagnose an aneurysm in an adult with repaired CoA?

Answer 120

3D imaging via CT or MRI (echo can often miss large aneurysms)

Question 121

Besides screening for aneurysm at the site of a CoA repair, what else is CTA or MRI helpful for in these patients?

Answer 121

Assessment of the size of the ascending aorta (especially if the patient has a bicuspid aortic valve)

Question 122

Current guidelines suggest surgical intervention for aneurysm of the ascending aorta once it is above what measurement?

Answer 122

>5-5.5cm

Question 123

What surgical approach is an option for patients who have a large ascending aortic aneurysm, but a well- functioning aortic valve (even if bicuspid)?

Answer 123

Valve-sparing aortic root replacement

Question 124

What is a big problem for patients who have undergone CoA repair (especially in those whose repair happens outside of infancy?

Answer 124

HTN

Question 125

Persistent HTN may predispose someone with repaired CoA to what?

Answer 125

Early atherosclerotic heart disease *Need to try to achieve goal directed medical therapy

Question 126

All adults with repaired CoA should undergo what to evaluate for aneurysm or re-coarctation at least every 5 years?

Answer 126

3D imaging of aorta

Question 127

What are options for 3D imaging of the aorta?

Answer 127

CT or MRI- Echo is helpful as a screening tool, but isn't good to detect things like aneurysm (especially in repaired CoA patients)

Question 128

What is a common problem in patients who have undergone repair of CoA?

Answer 128

HTN- Treatment needs to be aimed at goal-directed medical therapy

Question 129

True or False: Routine stress tests are part of the follow-up assessment of patients who have CoA?

Answer 129

False

Question 130

What is in the differential diagnosis for new-onset LV dilation and systolic dysfunction in a teenager?

Answer 130

-CHD -Cardiomyopathy -Myocarditis -Athletes heart

Question 131

What should be assessed during history for a teenager with new-onset LV dilation and systolic dysfunction?

Answer 131

-Recent viral illness (myocarditis) -Palpitations (tachyarrhythmia-induced cardiomyopathy) -Chest pain or syncope with exertion (coronary anomaly) -Exercise intolerance -Family history: Inherited forms of cardiomyopathy (dilated, arrhythmogenic, hypertrophic, LVNC) or CHD

Question 132

In pediatrics, athlete's heart is most often encountered in patients with what?

Answer 132

LVH and a questionable diagnosis of HCM

Question 133

True or False; Athlete's heart can overlap with other cardiomyopathies (DCM, arrhythmogenic cardiomyopathy, myocarditis)?

Answer 133

True

Question 134

True or False: Exercise-induced cardiac remodeling manifests differently in athletes in endurance v. strength training?

Answer 134

True

Question 135

Describe the exercise-induced cardiac remodeling seen in endurance athletes

Answer 135

-Volume overload during sustained high cardiac output -Biventricular dilation -Biatrial enlargement -Low normal resting EF -Mild-moderate eccentric LVH

Question 136

What causes the eccentric hypertrophy seen in endurance athletes?

Answer 136

New sarcomeres are added in series to existing sarcomeres leading to volume overload-induced cardiac hypertrophy *Distinct from pathologic hypertrophy in hypertrophic cardiomyopathy

Question 137

True or False: There are dilation volume cutoffs for the LV and RV which reliably differentiate between physiologic v. pathologic heart dilation?

Answer 137

False- Unless the heart is markedly dilated

Question 138

True or False: As little as 3-5 hours of exercise per week and >5 hours of exercise are associated with a high proportion of adults outside published normal ranges for both hypertrophy and dilation?

Answer 138

True

Question 139

True or False: In athletes, EFs are often lower than one would expect at rest given dilation of the LV?

Answer 139

True

Question 140

What is one way to distinguish a low-normal EF as being due to athletic status v. pathology?

Answer 140

Exercise test -Athletes wouldn't have exercise intolerance would probably have supranormal results on exercise testing without inducible arrhythmia -Imaging at rest would show normal diastolic parameters -Exercise imaging can show normalization of cardiac function

Question 141

Athlete's heart is a diagnosis of...

Answer 141

Exclusion (due to heightened awareness of SCD in young athletes) *Need to consider athlete's heart when factoring in exercise restriction... athletes with no pathologic disease can affect quality of life and economic opportunities

Question 142

What can be done to distinguish between athletes heart and cardiomyopathy?

Answer 142

-Period of deconditioning (3-6 months) to see if parameters normalize -If they don't, findings are consistent with cardiomyopathy v. athlete's heart

Question 143

What are some forms of CHD that may be associated with ventricular dilation and decreased function over time?

Answer 143

-L-R shunt lesions (ASD, VSD, PDA, AVM) -L obstructive lesions (CoA) -LV volume overload (valvulopathy) -Coronary anomalies *These will often have associated PE findings except coronary anomalies *Most picked up by echo, may need MRI or CT for something like sinus venosus ASD, coronary anomalies, aortic arch issues, etc

Question 144

True or False: Findings of athlete's heart can overlap with the various cardiomyopathies?

Answer 144

True- Dilated, hypertrophic, ARVC, myocarditis

Question 145

Which form of heterotaxy has associated twin AV nodes and SVT

Answer 145

Right atrial isomerism

Question 146

Which form of heterotaxy has associated complete heart block and bradyarrhythmias?

Answer 146

Left atrial isomterism

Question 147

True or False: All patients with heterotaxy syndrome are at risk for both tachy and bradyarrhythmias?

Answer 147

True

Question 148

What is the differential diagnosis when there is a change in QRS morphology on ECG?

Answer 148

-Intermittent preexcitation -Aberrant conduction -Ventricular rhythm -Twin AV nodes

Question 149

What is suggested when there is a change in QRS morphology always preceded by a change in the P-wave morphology?

Answer 149

Twin AV nodes *When the atrial impulse occurs from one site, it conducts down the close AV node *When the atrial stimulus occurs from a different site closer to the other AV node, conduction changes to that second AV node

Question 150

What are clues that change in QRS morphology is due to intermittent preexcitation?

Answer 150

-Usually no change in P-wave morphology that precedes each change in QRS morphology -Beats that are preexcited would have the characteristics of preexcitation (short PR, wide QRS, delta wave)

Question 151

What can occur with premature atrial beats that come at a time when one of the bundles is refractory?

Answer 151

Aberrant conduction

Question 152

What is rate-related aberrancy?

Answer 152

As the HR increases one of the bundles is refractory and not able to conduct

Question 153

What situations can result in aberrancy?

Answer 153

Acidosis Electrolyte abnormalities *QRS complex would gradually change not be a beat to beat difference

Question 154

True or False: There is usually not a change in P-wave morphology with aberrant conduction?

Answer 154

True

Question 155

What is the differential diagnosis for a change in QRS morphology?

Answer 155

-Intermittent preexcitation -Aberrant conduction -Ventricular rhythm -Twin AV nodes

Question 156

What is the differential diagnosis of a wide QRS tachycardia?

Answer 156

-Ventricular rhythm (v-tach, torsades) -Supraventricular rhythm with aberrancy (sinus tachycardia with underlying BBB or pre-excitation, SVT with aberrancy) -Antidromic supraventricular tachycardia -Artifact

Question 157

How can you distinguish sinus rhythm with artifact?

Answer 157

-See if QRS complexes can be detected within the artifact recording... will see narrow QRS complexes marching through the time of the artifact at a rate similar to what was seen before/after artifact -Can also look at other leads as well

Question 158

What is considered a toxic digoxin level?

Answer 158

>2ng/mL

Question 159

Generalized ST segment depression, T-wave inversion, accelerated junctional rhythm with atrioventricular block, curved sagging of ST segments?

Answer 159

Digoxin overdose

Question 160

True or False: Digoxin toxicity can cause nearly every possible arrhythmia?

Answer 160

True

Question 161

What are some arrhythmias that are more suggestive of digoxin toxicity?

Answer 161

-New-onset AV block -Accelerated junctional rhythm with or without high-degree AV block -Nonparoxysmal atrial tachycardia with AV block -Bidirectional ventricular tachycardia

Question 162

What will happen if digoxin toxicity is not promptly recognized and treated?

Answer 162

-Ventricular fibrillation -May not be responsive to defibrillation

Question 163

What is lifesaving in cases of digoxin toxicity?

Answer 163

Digoxin-specific antibody fragements

Question 164

What can be given for ventricular tachyarrhythmias due to digoxin toxicity?

Answer 164

Lidocaine or phenytoin (while awaiting digoxin antibodies)

Question 165

What can be given for bradyarrhythmias due to digoxin toxicity?

Answer 165

Atropine (while awaiting digoxin antibodies)

Question 166

What are common symptoms seen in digoxin toxicity?

Answer 166

GI- Diarrhea and vomiting

Question 167

What lab abnormality is seen in digoxin toxicity?

Answer 167

Hyperkalemia (indicative of severe digoxin toxicity)

Question 168

How does digoxin toxicity cause hyperkalemia?

Answer 168

-Blocks the sodium and potassium adenosine triphosphatase pump -Results in increased extracellular potassium that causes hyperkalemia

Question 169

What lab derangement can occur once antidigoxin antibodies are given for digoxin toxicity?

Answer 169

Hypokalemia- requires prompt correction

Question 170

Coadministration of what results in an increase in serum digoxin levels?

Answer 170

Amiodarone

Question 171

How is digoxin excreted?

Answer 171

Via kidneys through glomerular filtration and active tubular secretion

Question 172

Why does amiodarone result in increased serum digoxin levels?

Answer 172

It likely inhibits renal tubular secretion of digoxin

Question 173

What needs to be done to the digoxin dose in patients who are concomitantly using amiodarone?

Answer 173

Lower by 50% *Need to monitor digoxin levels carefully

Question 174

What can happen if amiodarone is started without lowering digoxin dose?

Answer 174

Digoxin toxicity

Question 175

An increase in what lab value may result in increased digoxin levels?

Answer 175

Creatinine- Digoxin is excreted by the kidneys, reduced kidney function could increase levels

Question 176

The ingestion of what dose of digoxin by a child without underlying heart disease can result in serious toxic effects?

Answer 176

0.3mg/kg

Question 177

True or False: Measurement of serum digoxin concentrations in kids doesn't always correlate with toxic effects?

Answer 177

True- Need a high index of suspicion and familiarity with the clinical signs and ECG changes

Question 178

True or False: Once antidigoxin antibodies are administered, serum digoxin levels can't be relied on and a 2nd dose may be needed after 12 hours based solely on clinical findings?

Answer 178

True

Question 179

When should extracorporeal cardiopulmonary resuscitation be considered?

Answer 179

In refractory cardiac arrest for select populations (children with heart disease) with a witnessed in-hospital cardiac arrest in settings with adequate systems and resources to use ECPR effectively

Question 180

True or False: On the basis of current studies, there is insufficient evidence to recommend consideration of extracorporeal cardiopulmonary resuscitation for out-of-hospital cardiac arrest?

Answer 180

True *Indications rapidly evolving and individual patients considerations should be discussed with the responsible ICU, ECMO and surgical teams before determining any specific patient's eligibility

Question 181

Can patients in the ICU for noncardiac conditions with witnessed arrest be considered for extracorporeal cardiopulmonary resuscitation?

Answer 181

Yes... but patients with things like septic shock are unlikely to receive adequate flow/supports through peripheral cannulation and would have less favorable outcomes (unless surgeon would do central cannulation)

Question 182

What does extracorporeal cardiopulmonary resuscitation involve?

Answer 182

Adjunctive use of mechanical cardiopulmonary support to assist during or immediately after cardiopulmonary resuscitation

Question 183

When should extracorporeal cardiopulmonary resuscitation be considered?

Answer 183

In refractory cardiac arrest for select populations (children with heart disease) with a witnessed in-hospital cardiac arrest in settings with adequate systems and resources to use ECPR effecitvely

Question 184

What are commonly used benzodiazepines in pediatric cardiac patients?

Answer 184

-Lorazepam -Diazepam -Midazolam

Question 185

How do benzodiazepines work?

Answer 185

Bind to the GABA (y-aminobutyric acid) A receptors, mostly in the CNS, and hyperpolarizing them to be more responsive to GABA inhibition -Leads to sedation, anxiolysis and decreased excitability

Question 186

What are the effects of benzodiazepines?

Answer 186

Sedation, anxiolysis, decreased excitability

Question 187

What causes the cardiac effects of benzodiazepiens?

Answer 187

Combination of reduced sympathetic activity caused by sedation and reduced parasympathetic stimulation in response to GABA activation in the nucleus ambiguus and reduced vagal tone

Question 188

What are the cardiac effects of benzodiazepines?

Answer 188

-Depends on dose -Hypotension -Bradycardia or tachycardia: Attributable to reduction in sympathetic stimulation or overinhibition of parasympathetic outflow and baroreceptor reflex stimulation by lowered BP -Direct negative inotropic effect on myocardial contractility (due to Ca channel suppression in the SR)

Question 189

True or False: Benzodiazepines are generally well tolerated at low doses?

Answer 189

True

Question 190

True or False: Benzodiazepines have been directly implicated in acute CV collapse and cardiac arrest (mostly at high doses), in rapid IV bolus, or in neonates/patients with cyanotic CHD?

Answer 190

True- Use with caution

Question 191

What is a benzodiazepine antagonist?

Answer 191

Flumazenil

Question 192

How does flumazenil work?

Answer 192

Competitive inhibition at the benzodiazepine-binding site on GABA receptors *Used as an antidote in benzodiazepine overdose

Question 193

What causes the cardiac effects of flumazenil?

Answer 193

Increased sympathetic stimulation with benzodiazepine reversal

Question 194

What are the cardiac effects of flumazenil?

Answer 194

-Tachycardia -Ventricular ectopy -SVT *Older adults, can see complete heart block and spontaneous cardiac arrest, but not understood why this happens

Question 195

What happens to patients that receive flumazenil?

Answer 195

-Transient increase in circulating epinephrine and norepinephrine -Results in increased HR, BP, PA pressure, wedge pressure and LVEF -No significant increase in CVP

Question 196

What are the non-cardiac effects of flumazenil?

Answer 196

-Seizures or status epilepticus (can be fatal) -Anxiety -Agitation

Question 197

Which patients have the most concerning side effects from flumazenil?

Answer 197

Those with longstanding benzodiazepine dependence (seizures and status epilepticus which can be fatal)

Question 198

What can happen if a patient has had a large dose of benzodiazepine and was then given flumazenil?

Answer 198

Can develop rebound sedation after antagonistic effects of flumazenil wear off

Question 199

What causes benzodiazepines effects on cardiac activity?

Answer 199

-Reduced parasympathetic outflow (reducing vagal tone) -Reduced sympathetic stimulation -Direct myocardial suppression

Question 200

What are benzodiazepine antagonist effects on cardiac activity attributable to?

Answer 200

Increased sympathetic outflow causing a transient increase in circulating epinephrine and norepinephrine levels

Question 201

True or False: Benzodiazepines and their antagonists must be used with extreme caution in patients with cyanotic CHD, neonates and those with abnormal cardiac function?

Answer 201

True

Question 202

Grade 2/6 systolic murmur along the left sternal border that becomes louder when rising from a crouched position?

Answer 202

LVOT murmur (think HOCM)

Question 203

What are some ECG findings consistent with HOCM?

Answer 203

-Sinus bradycardia -Pronounced ventricular hypertrophy by voltage -T-waves in precordial leads with a strain pattern

Question 204

What is the Sokolow-Lyon criteria for ventricular hypertrophy?

Answer 204

Sum of the R wave in V6 and S wave in V1 >35mm

Question 205

True or False: LVH on resting ECG has a low sensitivity/specificity and PPV for LVH?

Answer 205

True

Question 206

What should you consider for an ECG with deep S wave in V6, tall R wave in V1 and t-wave inversion in the lateral leads?

Answer 206

-Pulmonary HTN -PS -Other pathologies involving RV and outflow tract

Question 207

What is the modified Bernoulli equation?

Answer 207

Pressure Gradient (mmHg) = 4V2 (V = peak velocity)

Question 208

In the modified Bernoulli equation, what is ingored?

Answer 208

The proximal velocity- ignored if it is <1 M/sec *When a fixed or dynamic proximal gradient is present, the accelerated velocity must be factored into the equation

Question 209

True or False: In the setting of severe multilevel obstruction, the presence of 2 stenoses in series may make it impossible to ascertain the precise individual contribution of each by echo?

Answer 209

True

Question 210

Which tends to be higher, Doppler peak gradients or peak-to-peak cath gradients?

Answer 210

Doppler peak gradients

Question 211

What constitutes mild pulmonary valve stenosis?

Answer 211

Peak velocity <3M/sec Peak gradient <36mmHg

Question 212

What constitutes moderate pulmonary valve stenosis?

Answer 212

Peak velocity 3-4M/sec Peak gradient 36-64mmHg

Question 213

What constitutes severe pulmonary valve stenosis?

Answer 213

Peak velocity >4M/sec Peak gradient >64mmhg

Question 214

An RV pressure of what % systemic is consistent with moderate pulmonary stenosis?

Answer 214

50-75%

Question 215

True or False: Severity of PS using Doppler derived gradients has demonstrated a reasonable correlation between peak-to-peak gradients obtained by catheterization?

Answer 215

True

Question 216

How can you calculate pressure decrease across a stenotic semilunar valve by echo?

Answer 216

Peak velocities obtained from CW Doppler signals across a stenotic semilunar valve are converted into a pressure gradient by the modified Bernoulli equation

Question 217

What are other names for a doubly committed juxta-arterial VSD?

Answer 217

-Subpulmonary -Supracristal -Subarterial

Question 218

Where is a double committed juxta-arterial VSD seen on a short axis view on echo?

Answer 218

-Proximal to the pulmonary valve and more distal from the tricuspid valve *Perimembranous VSD are seen in close proximity to the tricuspid valve

Question 219

What can be used to estimate the pressure gradient between the right and left ventricles in patients with VSDs?

Answer 219

CW Doppler

Question 220

How do you estimate the pressure gradient between the RV and LV in a patient with a VSD?

Answer 220

*Doppler measurement of peak velocity across defect *Convert to pressure gradient using modified Bernoulli equation (P = 4V2 *Subtract pressure gradient from patient's SBP to estimate RVSP and PA pressure

Question 221

True or False: In the absence of RVOTO, smaller VSDs are nearly always associated with restrictive hemodynamics?

Answer 221

True- High velocity and high peak trans-septal gradient with low estimated RV pressures

Question 222

What type of VSDs tend to demonstrate unrestrictive hemodynamics?

Answer 222

Large VSD *Low velocity and low peak trans-septal gradient with elevated estimated RV pressures

Question 223

What causes a doubly committed juxta-arterial VSD?

Answer 223

Absence of the subpulmonary infundibulum (this normally serves to lift the pulmonary trunk away from the base of the heart) *Results in fibrous continuity between the aortic and pulmonary valves

Question 224

What can happen to the aortic valve in a doubly committed juxta-arterial VSD?

Answer 224

Risk for aortic valve leaflet prolapse (most commonly the right coronary cusp) due to deficiency of muscular support

Question 225

What should be done for a small, restrictive, doubly committed juxta-arterial VSD?

Answer 225

-Supportive care -Routine follow-up to assess for future evidence of aortic valve prolapse or AI

Question 226

What information does Spectral Doppler provide about VSD hemodynamics?

Answer 226

Characterizes restrictive v. nonrestrictive based on the peak velocity by using the modified Bernoulli equation to estimate peak trans-septal pressure gradients

Question 227

Why do doubly committed juxta-arterial VSDs need to be monitored so closely?

Answer 227

Risk for AV prolapse

Question 228

What defines critical PS?

Answer 228

Ductal dependence to maintain adequate pulmonary blood flow

Question 229

What is the treatment of choice for valvar PS?

Answer 229

Pulmonary balloon valvuloplasty

Question 230

What might be needed following pulmonary balloon valvuloplasty?

Answer 230

PGE

Question 231

Why might an infant need PGE following pulmonary balloon valvuloplasty?

Answer 231

RVH associated with poor compliance and a hyperdynamic infundibulum can result in preferential R-L atrial shunting of systemic venous return and a reduced RV output

Question 232

What can be given after pulmonary balloon valvuloplasty to improve diastolic filling of the noncompliant, hypertrophied and potentially hypercontractile RV?

Answer 232

Beta blockade

Question 233

How does beta blockade help after pulmonary balloon valvuloplasty?

Answer 233

-Reduces HR -Reduces contractility -Improves diastolic filling *This can promote antegrade flow across the pulmonary valve

Question 234

What kind of receptors are beta-adrenergic receptors and where are they located?

Answer 234

-Transmembrane guanine nucleotide (G) protein-coupled receptors -Located on the cardiac sarcolemma

Question 235

What activates B-adrenergic receptors?

Answer 235

-Norepinephrine -Epinephrine -Catecholamines

Question 236

Stimulatory (Gs) proteins link G-protein coupled receptors to what, which then produces what?

Answer 236

-Adenylyl cyclase -Cyclic adenosine monophosphate (cAMP) from adenosine triphosphate

Question 237

How do inhibitory (Gi) protein receptors inhibit the production of cAMP?

Answer 237

Deactivate adenylyl cyclase

Question 238

What does cAMP activate that results in an increase in the rate/force of myocardial contraction through the opening of Ca channels and increase in myocardial relaxation through increased reuptake of Ca?

Answer 238

Protein kinase A pathway

Question 239

How does Gs protein receptor stimulation affect conduction?

Answer 239

-Accelerates rate of conduction -Gs protein receptor stimulation within the conduction system increases the pacemaker current within the sinus node... accelerates rate of conduction

Question 240

What interrupts the link between G-protein coupled receptors and adenylyl cyclase resulting in decreased contractility and HR?

Answer 240

Muscarinic stimulation of Gi protein receptors after vagal activation

Question 241

What improves with a reduction in HR?

Answer 241

Filling time- there is an increase in the amount fo time spent in diastole during the cardiac cycle

Question 242

Where are B1 adrenergic receptors found?

Answer 242

Heart

Question 243

Where are B2 adrenergic receptors found?

Answer 243

Bronchial and vascular smooth muscle

Question 244

What do B-blockers result in?

Answer 244

-Reduction in rate/force of myocardial contraction -Reduction in pacemaker current and rate of conduction

Question 245

What is the difference between first, second and thirs generation B-blockers?

Answer 245

-First generation are nonselective for B1/B2 blockade -Second generation have a relative selectivity for B1 -Third are nonselective with additional benefits like alpha-blocking activity

Question 246

Propranolol and nadolol are what generation B-blocker?

Answer 246

1st

Question 247

Metoprolol, atenolol and esmolol are what generation B-blocker?

Answer 247

2nd

Question 248

Carvedilol is what generation B-blocker?

Answer 248

3rd

Question 249

What are 2 proposed mechanisms for Beta-blockers decreasing BP?

Answer 249

-Reduced CO -Blockade of B-receptors within the renal juxtaglomerular complex (reduces circulation levels of renin)

Question 250

What causes the reduction in myocardial O2 demand for patients taking a B-blocker?

Answer 250

-Reduction in HR and BP -Limiting increases in contractility that result from exercise or agitation

Question 251

What are the effects of B-blockers on the heart?

Answer 251

-Reduction in rate and force of myocardial contraction -Reduction in pacemaker current and rate of conduction

Question 252

Elevated partial pressure of PCO2 with proportionately low pH?

Answer 252

Acute respiratory acidosis without compensation

Question 253

In acute onset respiratory acidosis, the pH decreases by 0.08 for every _mmHg increase in PCO2 above normal (40mmHg)?

Answer 253

10

Question 254

In the case of chronic respiratory acidosis (>72 hours), metabolic compensation with an increase in serum bicarbonate is predicted by what formula?

Answer 254

3.5 * (change in PCO2/10)

Question 255

What type of ventilation is optimal for Glenn circulation and why?

Answer 255

-Negative pressure -Extubated -Spontaneous breathing -SVC blood return to the pulmonary vasculature is passive

Question 256

What are the effects of mild hypercapnia (PCO2 45-55mmHg) on Glenn physiology?

Answer 256

-Leads to cerebral vasodilation -Leads to increased cerebral blood flow -May improve perfusion through the Glenn circulation

Question 257

Giving what can improve metabolic acidosis or a pulmonary HTN crisis?

Answer 257

NaBicarb

Question 258

If a patient doesn't have adequate ventilatory capacity, giving what can worsen hypercapnia and acidosis, potentially leading to pulmonary vasoconstriction?

Answer 258

Bicarb

Question 259

What should be done to the ventilator settings if the lungs are overinflated or there is inadequate exhalation (like in bronchospasm)?

Answer 259

Decrease PEEP

Question 260

Worsening compliance can lead to what metabolic derangement?

Answer 260

Respiratory acidosis

Question 261

What should always be considered in cases of respiratory acidosis?

Answer 261

Malpositioned ET tube

Question 262

Permissive hypercapnia up to what improves pulmonary blood flow in the Glenn circulation?

Answer 262

55mmHg *But hypercapnia leading to acidosis may also trigger pulmonary vasoconstriction

Question 263

What defines tricuspid atresia?

Answer 263

-Cyanotic CHD -Complete absence of tricuspid valve, obligate R-L atrial level shunting, hypoplasia of RV

Question 264

What do the clinical features in tricuspid valve atresia largely depend on?

Answer 264

-Great artery relationship -Presence or absence of obstruction to pulmonary blood flow or systemic flow

Question 265

True or False: Most patients with tricuspid atresia have normally related great arteries?

Answer 265

True

Question 266

What % of patients with tricuspid atresia have D-TGA?

Answer 266

12-25% *Aorta comes from hypoplastic RV

Question 267

In tricuspid atresia, what does a large VSD in the setting of normally related great arteries result in?

Answer 267

Overcirculation

Question 268

In tricuspid atresia, what does a restrictive VSD, pulmonary stenosis or pulmonary atresia result in?

Answer 268

Reduced pulmonary flow and ductal dependency

Question 269

Tricuspid atresia with D-TGA and restrictive VSD result in what?

Answer 269

Compromised systemic blood flow- may be associated with a hypoplastic aortic arch or CoA

Question 270

True or False: All patients with tricuspid atresia are committed to the single ventricle pathway?

Answer 270

True -Goal to transition from parallel systemic and pulmonary circulations to circulations that are in series

Question 271

What will happen to a baby with time who has tricuspid atresia with normally related great vessels, a large VSD and no evidence of obstruction to pulmonary blood flow?

Answer 271

Will develop excessive pulmonary blood flow as PVR drops- may be tachypneic, etc

Question 272

What should be done for an infant with tricuspid atresia with normally related great vessels, a large VSD and no evidence of obstruction to pulmonary blood flow?

Answer 272

-Begin diuretic therapy -Consider MPA band to protect pulmonary vascular bed from excessive pulmonary blood flow/high pressure (this makes sure the patient will be a candidate for the bidirectional Glenn then Fontan)

Question 273

Why do patients need to be monitored for cyanosis after PA band in the setting of tricuspid atresia with normally related great vessels?

Answer 273

-Ventricular hypertrophy can develop -This can lead to restriction of the ventricular level shunt

Question 274

What needs to be done for an infant with tricuspid atresia, normally related great arteries and reduced pulmonary blood flow due to a restrictive VSD, PS or PA?

Answer 274

-Need a L-R shunt -PGE to maintain ductal patency -Eventual systemic to PA shunt or ductal stent for stable source of pulmonary blood flow

Question 275

A DKS (MPA to ascending aorta) with systemic to PA shunt is needed in tricuspid atresia with what?

Answer 275

D-TGA and restrictive VSD -Will have restricted systemic blood flow from subaortic obstruction from VSD and pulmonary overcirculation from increased CO from LV to MPA -May need ductal patency to ensure adequate systemic output until surgery

Question 276

Patients with tricuspid atresia and what will experience pulmonary overcirculation and will benefit from PA banding?

Answer 276

-Normally related great vessels -Large VSD

Question 277

What type of tricuspid atresia patients are ductal dependent pending establishment of a stable source of pulmonary blood flow?

Answer 277

-Normally related great arteries -Reduced pulmonary blood flow due to restrictive VSD, PS, or PA

Question 278

What type of tricuspid atresia patient will have restrictive systemic blood flow and pulmonary overcirculation?

Answer 278

-D-TGA -Restrictive VSD

Question 279

What is needed for tricuspid atresia patients with D-TGA and restrictive VSD?

Answer 279

-DKS with systemic to PA shunt (ensures adequate systemic blood flow with reduced low pressure pulmonary flow) *Hypoplastic arch or CoA can also be addressed

Question 280

When does subaortic stenosis most often occur?

Answer 280

In association with other forms of CHD or after prior surgical repair of CHD (rare as an isolated defect)

Question 281

What are the most common congenital cardiac anomalies associated with subaortic stenosis?

Answer 281

-VSD -PDA -Bicuspid aortic valve -CoA -IAA (especially with co-existing Shone syndrome -AVCD

Question 282

What can be seen years following repair of a posterior malalinged VSD and IAA?

Answer 282

LVOTO due to subaortic membrane

Question 283

What is IAA usually associated with?

Answer 283

-Posterior malalignment of the infundibular ventricular septum with a VSD in that region -Aortic valve hypoplasia (bicuspid) -AS (can be significant)

Question 284

What does neonatal repair of IAA typically include?

Answer 284

-VSD patch closure -Aortic arch reconstruction

Question 285

What are options for neonatal repair of IAA + VSD in patients where primary VSD closure may not address the LVOTO (or LVOT stenosis or AS are significant)?

Answer 285

-Resection of infundibular septum with VSD enlargement -Single or staged repair via Norwood-Rastelli approach (depending on degree of LVOT narrowing and AV morphology) -Single ventricle palliation (severe LVOTO)

Question 286

True or False: Patients with interrupted aortic arch/VSD often have residual and/or recurrent LVOTO?

Answer 286

True- may need to be re-addressed surgically

Question 287

True or False: It would be unusual for a neonate to require isolated AV canal repair?

Answer 287

True (usually done at a few months of age)

Question 288

True or False: Subaortic membrane is not a common post-operative complication after TAPVR?

Answer 288

True

Question 289

What is a common complication after IAA/VSD repair?

Answer 289

Subaortic stenosis

Question 290

What infants are considered high-risk and should be referred to a neurodevelopmental follow-up program?

Answer 290

-Exposure to postnatal corticosteroids -TPN use -In utero alcohol, nicotine or controlled substances -High-risk cyanotic lesions -Prolonged critical illness -Neonatal surgery with CPB -Prematurity -Developmental delay -Suspected syndrome or genetic anomaly -Mechanical circulatory support -Transplant -Cardiopulmonary resuscitation -Seizures -Abnormal neuroimaging *Does not specifically list high-risk medications

Question 291

What are the most commonly implicated neonatal medication exposures associated with neurodevelopmental disorders?

Answer 291

-Neuro-sedative agents -Antiepileptics -Glucocorticoids

Question 292

What are some of the adverse effects of loop diuretics (furosemide and bumetanide)?

Answer 292

-Hypokalemia -Hypochloremia -Metabolic alkalosis -Hypercalciuria (leads to bone loss/renal calcification with chronic use) -Ototoxicity

Question 293

What is the ototoxic effect of loop diuretics thought to be related to?

Answer 293

-Peak serum concentration reaching a toxic level *Recent study reported complete elimination of sensorineural hearing loss in patients with single ventricle disease after QI slowing the infusion rate of IV loop diuretics (lower peak serum concentration at slower rates)

Question 294

True or False: Bumetanide has significantly less ototoxicity than furosemide at equivalent diuretic doses?

Answer 294

True- even though it has a similar MOA on the cochlea

Question 295

True or False: The ototoxic effects of loop diuretics may be delayed?

Answer 295

True

Question 296

Why is it important for patients who have required loop diuretics to have good neurodevelopmental follow-up?

Answer 296

-Risk for ototoxicity and sensorineural hearing loss -Ototoxic effects may be delayed -Many patients discharged home on diuretics -Need comprehensive neurodevelopmental follow-up including interval language development assessments

Question 297

True or False: In-utero exposure to acetaminophen has been associated with autism and ADHD?

Answer 297

True *But no data linking postnatal acetaminophen with neurodevelopmental disorders

Question 298

True or False: No studies have independently associated prostaglandins or ranitidine with neurodevelopmental delays?

Answer 298

True

Question 299

What patients should be referred to a comprehensive neurodevelopmental follow-up program?

Answer 299

-Complex CHD -Risk factors for neurodevelopmental disorders: Exposures to antiepileptics, neurosedatives, high-dose loop diuretics, corticosteroids

Question 300

What is the position of the MPA relative to the aorta in D-TGA?

Answer 300

Posterior and leftward to the aorta

Question 301

A large LA, elevated E/A ratio and short MV deceleration time are all indicative of what?

Answer 301

Abnormal diastolic function

Question 302

Chronic LA HTN due to restrictive physiology can result in what?

Answer 302

-Arrhythmias -Pulmonary HTN -Thromboembolic events (stasis or pooling of blood in atria *Could all potentially result in a syncopal episode

Question 303

What happens to patients with restrictive physiology during exercise?

Answer 303

-Increased HR can help increase CO up to a point -These patients have a relatively fixed SV which decreases ability to compensate for increased demands of exercise (ventricles fill less well with elevated HR)

Question 304

True or False: LAE in the absence of significant MR is a relatively good marker for diastolic dysfunction in children?

Answer 304

True

Question 305

Describe the findings on mitral valve inflow Doppler that are consistent with restrictive physiology

Answer 305

-Tall, narrow mitral E wave -Diminutive A wave -E/A ratio >3 -E wave deceleration time short (<130ms)

Question 306

What causes the short E-wave deceleration time on mitral inflow Doppler in restrictive physiology?

Answer 306

Rapid equalization of the LA filling pressure with the elevated LVEDP in a stiff, less compliant ventricle

Question 307

True or False: No single echo parameter for diastolic function should be used in isolation as a determiner for the presence of diastolic dyusfunction?

Answer 307

True

Question 308

True or False: The application of adult echo criteria for diastolic dysfunction has overall poor correlation in pediatric patients?

Answer 308

True

Question 309

What are the more useful parameters in children for the assessment of diastolic dysfunction?

Answer 309

-LA volume/size -Short mitral deceleration times

Question 310

What gives the most accurate assessment of the ventricular filling pressures and quantification of diastolic abnormalities (including restrictive physiology)?

Answer 310

Direct measurement of LVEDP via cath *Also gives PA pressures and PVR which can become significantly elevated with restrictive physiology

Question 311

What are some of the risks associated with cardiac cath?

Answer 311

-Myocardial perforation -Arrhythmia -TV or vessel injury

Question 312

What findings would you expect on cath for a patient with a tall, narrow mitral E wave, diminutive A wave, E/A ratio >3 and E wave deceleration time <130msec on echo?

Answer 312

Elevated LVEDP (restrictive physiology)

Question 313

Despite preserved systolic function on echo, patients after aortic valve interventions are at risk for what?

Answer 313

Diastolic dysfunction (restrictive physiology being on severe end of spectrum)

Question 314

True or False: All patients with CHD should be educated about their diagnosis and the reasons they will need lifelong cardiology care?

Answer 314

True

Question 315

What are some of the life-long risks for patients with CoA who have undergone surgical or catheter-based repair?

Answer 315

-Recurrent CoA -Aortic aneurysms -Pseudoaneurysms -Aortic dissection -HTN

Question 316

What % of patients with repaired CoA require reintervention in adulthood?

Answer 316

11%

Question 317

What kind of imaging should all adults with repaired CoA have periodically?

Answer 317

3D imaging of the aorta via MRA or CTA

Question 318

What are some of the Class I recommendations for adults with repaired CoA?

Answer 318

-3D imaging of aorta via MRA or CTA periodically -Checking resting BP in upper/lower extremities to assess for recoarctation -Surgical repair or catheter-based stenting in patients with recoarctation and HTN -Goal-directed medical therapy for the management of HTN

Question 319

When treating an adult s/p CoA repair for HTN, what do you have to be cautious about?

Answer 319

That degree of CoA isn't so severe to acutely decrease LE perfusion with aggressive anti-hypertensives until recurrent CoA is managed appropriately

Question 320

What are the Class II recommendations for adults with repaired CoA?

Answer 320

-Ambulatory BP monitoring to diagnose/manage HTN -Screening for intracranial aneurysms -Exercise testing to assess for exercise-induced HTN -Balloon angioplasty in patients with CoA who are ineligible for stent placement or surgical repair

Question 321

Intracranial aneurysms can occur in up to what % of patients with CoA?

Answer 321

10%- and can increase in frequency with age

Question 322

True or False: In adults s/p CoA repair, TEE may be able to identify an area of recurrent CoA, but can't profile the entire thoracic aorta and may be insufficient for identifying aneurysms, pseudoaneurysms, dissections or remotely located CoA?

Answer 322

True- why cross-sectional imaging is more appropriate (especially with incomplete history or gaps in follow-up)

Question 323

What is the most common genetically inherited hyperlipidemia?

Answer 323

Familial hypercholesterolemia

Question 324

Which is more common, heterozygous or homozygous familial hypercholesterolemia?

Answer 324

Heterozygous -Heterozygous: 1/200-250 -Homozygous: 1/160,000-300,000

Question 325

When should children be screened for hyperlipidemia regardless of risk factors or comorbidities?

Answer 325

9-11 and 17-21

Question 326

Which children should have hyperlipidemia screening starting after age 2?

Answer 326

-CV Risk Factors: CHD, Kawasaki disease with persistent coronary artery aneurysms, heart transplants -Non-CV Risk Factors: Chronic renal disease, DM, stem cell transplants

Question 327

Familial hypercholesterolemia should be suspected in any pediatric patient with what findings?

Answer 327

-Fasting LDL >160mg/dL -Family history of premature CAD or elevated LDL in a 1st degree relative

Question 328

If an LDL level > what is detected in a patient <20 years, there is an 80% chance of familial hypercholesterolemia?

Answer 328

190

Question 329

Mutations in what genes can be detected by genetic testing for familial hyperlipidemia?

Answer 329

-LDL receptor -Apolipoprotein B -Proprotein convertase subtilisin/kesin type 9 (PCSK9) *Genetic testing isn't routinely recommended

Question 330

What are the first line of pharmacologic agents to treat familial hypercholesterolemia in children and adults?

Answer 330

Statins

Question 331

How do statins work?

Answer 331

-Inhibition of the 3-hydroxy-3-methylglutaryl coenzyme A reductase (rate-limiting enzyme in cholesterol biosynthesis) -Upregulates LDL receptors in the liver

Question 332

Besides lowering LDL, what other effects do statins have?

Answer 332

-Modest effect on lowering triglyceride levels -Modest effect on raising high-density lipoprotein cholesterol levels

Question 333

What does the expected effect on LDL lowering with statin use depend on?

Answer 333

Dose and intensity of the statin

Question 334

What are considered to be the more potent statins?

Answer 334

-Atorvastatin -Rosuvastatin

Question 335

Which 2 statins are approved for use in children as young as 8?

Answer 335

-Pitavastatin -Pravastatin

Question 336

Which statins are approved for use in children over 10?

Answer 336

-Atorvastatin -Rosuvastatin -Lovastatin -Fluvastatin *In addition to pitavastatin and pravastatin

Question 337

How are statins started/titrated in children?

Answer 337

-Start with lowest possible dose -Gradual up-titration to achieve desired LDL <130

Question 338

What are some of the adverse effects of statins?

Answer 338

-Statin-associated muscle symptoms -Increase in hepatic transaminases -Transient GI upset -Concern for teratogenicity

Question 339

True or False: Multiple studies have found no adverse effects of statins on growth, development or sexual maturation?

Answer 339

True

Question 340

What are clinically significant adverse effects of statins that require discontinuation of therapy?

Answer 340

-Severe myopathy -Rhabdomyolysis -Hepatic dysfunction *Rare in children and adolescents

Question 341

How often should LFTs be checked when starting a statin?

Answer 341

-Before initiation -Repeat measurements at 4, 8 and 12 weeks after initiation

Question 342

An elevation in hepatic transaminases above what is concerning and should prompt discontinuation of statin therapy?

Answer 342

>3x upper limit of normal *Should evaluate for alternative causes for the increase as well

Question 343

True or False: If you stop a statin for elevated transaminases, you can re-initiate treatment with a statin at a lower dose (or different statin) with close monitoring of transamianses once the levels have normalized?

Answer 343

True

Question 344

Besides LFTs, what other lab value should be measures before initiating a statin?

Answer 344

Creatinine kinase (CK)

Question 345

When do repeat CK levels need to be checked when starting a statin?

Answer 345

Only if the patient has muscle symptoms

Question 346

A CK level above what should prompt temporary discontinuation of a statin?

Answer 346

CK >10x the upper limit of normal *or clinically significant statin-associated muscle symptoms- be sure to assess for alternative etiologies (exercise, hypothyroidism, trauma)

Question 347

True or False: You can try to reinitiate statin therapy after it was discontinued due to elevated CK/muscle symptoms?

Answer 347

True- once levels/symptoms have normalized... re-start in controlled fashion at a lower dose

Question 348

What do you need to counsel female patients about who are taking a statin?

Answer 348

Teratogenic effects of the drug

Question 349

What can be given to patients with clinically significant elevations in TG levels to prevent acute episodes of pancreatitis?

Answer 349

Fenofibrate or bezafibrate (minimal effect on LDL)

Question 350

What is a cholesterol absorption inhibitor that is used as an adjunct with statin for lowering LDL to reach the optimal levels?

Answer 350

Ezetimibe

Question 351

What can be used as monotherapy for LDL control in patients with adverse effects to statins?

Answer 351

Ezetimibe (but this is rare)

Question 352

How much does ezetimibe lower LDL?

Answer 352

By 15-20%

Question 353

What age and what dose can you use ezetimibe?

Answer 353

>10 years, 10mg/day

Question 354

What supplements can be used to lower LDL levels up to 15%?

Answer 354

Phytosterol

Question 355

What is the dose of phytoserol?

Answer 355

2g/day

Question 356

When is phytosterol supplement no effective?

Answer 356

When a reduction LDL >50% or up to <130mg/dL is desired *This is what's needed in patients with familial hypercholesterolemia and why statins are used

Question 357

What is the most common genetically inherited hyperlipidemia?

Answer 357

Familial hyoercholesterolemia

Question 358

What is the most common hertiable chest wall abnormality?

Answer 358

Pectus excavatum

Question 359

What is pectus excavatum?

Answer 359

Congenital anterior chest wall deformity in which the sternum and rib cage are shaped abnormally, leading to a funnel-shaped or caved-in appearance of the chest

Question 360

When does pectus excavatum become most noticeable?

Answer 360

During adolescent growth spurts (but can be present from birth)

Question 361

Pectus abnormalities are more common in which gender?

Answer 361

Males (by a factor of at least 2:1)

Question 362

What testing is usually done as part of the evaluation for possible pectus?

Answer 362

TTE

Question 363

Severe cases of pectus excavatum have been associated with what cardiac findings?

Answer 363

-RV distortion -RV compression -Impaired RV filling *These are considered a rationale for surgical repair beyond cosmetic reasons

Question 364

Pectus excavatum and carinatum are associated with what other findings?

Answer 364

Marfan syndrome and other connective tissue abnormalities (Loeys-Dietz and Ehlers-Danlos)

Question 365

Isolated pectus excavatum is associated with an increased risk for what?

Answer 365

MVP- reported in 18-45% of patients with isolated pectus abnormalities

Question 366

Besides MVP, what other finding is more common in patients with pectus excavatum than the general population?

Answer 366

Aortic root dilation

Question 367

Patients who have a combination of pectus excavatum (or carinatum), MVP and aortic dilation should undergo a thorough investigation for what?

Answer 367

Other connective tissue abnormalities (even if other physical stigmata of a connective tissue disorder aren't obvious)

Question 368

True or False: Many genetic abnormalities can be associated with pectus chest wall abnormalities and cardiac abnormalities (like Noonan and Turner)?

Answer 368

True

Question 369

True or False: Isolated pectus excavatum has been assocaited with abnormalities of teh RV including RV awll motion abnormalities due to compression or distortion, pulmonary hypertension and RV dysfunction?

Answer 369

False *Yes to RV wall motion abnormalities due to compression or distortion *No to pulmonary HTN or RV dysfunction

Question 370

What is the most common surgical technique for repair of isolated pectus excavatum abnormality?

Answer 370

Nuss procedure

Question 371

How is the Nuss procedure for repair of isolated pectus excavatum abnormality performed?

Answer 371

-Minimally invasive surgical thorascopy technique -Position a bar (Lorenz or Nuss bar) below the sternum -Then it is inverted to raise and reposition the sternum and ribcage

Question 372

What is warranted before possible surgical intervention for pectus excavatum?

Answer 372

Cardiology clearance to assess for evidence of cardiac distortion or compression (RV) in addition to MVP, aortic dilation or other forms of CHD that may distort or complicate the surgical intervention

Question 373

How long does a Nuss bar remain in place following repair of isolated pectus excavatum abnormality?

Answer 373

2-3 years, then removed

Question 374

True or False: After the Nuss procedure, improvement in cardiopulmonary performance has been reported in patients with pectus excavatum?

Answer 374

True

Question 375

Patients with pectus excavatum who are also found to have MVP, aortic root dilation or both should undergo what?

Answer 375

Genetic screening for possible associated connective tissue disorders such as Marfan

Question 376

What is cor triatriatum sinister?

Answer 376

-Congenital cardiac lesion in which the LA is divided into 2 chambers by a fibromuscular diaphragm -Diaphragm can result in obstruction of the pulmonary venous return depending on the presence and number of fenestrations in the diaphragm -Rare- 0.4% of patients with CHD

Question 377

What are the most commonly associated lesions with cor triatriatum sinister?

Answer 377

-ASD -Anomalous pulmonary venous connection

Question 378

When do symptoms with cor triatriatum sinister develop?

Answer 378

Usually infancy, but has been reported to be incidentally found in adulthood

Question 379

What are the symptoms of cor triatriatum sinister?

Answer 379

-Similar to MS -Dyspnea, poor weight gain, poor feeding

Question 380

When is surgery indicated for cor triatriatum sinister?

Answer 380

When there is obstruction at the membrane -Also repair any associated lesions

Question 381

True or False: Surgery for cor triatriatum sinister is considered safe, with a very low risk of recurrent obstruction?

Answer 381

True

Question 382

What is it called when there is cor triatriatum in the right atrium?

Answer 382

Cor triatriatum dexter

Question 383

What is caused by a fibromuscular diaphragm that separates the LA into 2 chambers?

Answer 383

Cor triatriatum sinister

Question 384

What are symptoms in cor triatriatum sinister related to?

Answer 384

Presence and degree of obstruction at fenestrations in the diaphragm

Question 385

What are symptoms of cor triatriatum sinister similar to?

Answer 385

MS- Tachypnea, poor growth, poor feeding

Question 386

How do you identify the location of an accessory pathway?

Answer 386

Find the earliest atrial activation during tachycardia

Question 387

For an EP study, the distal CS (CS1) is located where?

Answer 387

Most leftward (v. proximal CS (CS9) which is closer to the atrial septum)

Question 388

In patients with a right lateral accessory pathway, where would you expect the earliest atrial activation to be?

Answer 388

Around the high right atrium catheter

Question 389

For a patient with SVT using a septal accessory pathway, the earliest retrograde atrial activation would be where?

Answer 389

Around CS9 (proximal CS) or close to the His catheter

Question 390

Describe the circuit for SVT with an accessory pathway?

Answer 390

-Antegrade via AV node -Retrograde via AP

Question 391

Describe the circuit for SVT with typical AVNRT

Answer 391

-Down the slow -Up the fast

Question 392

For an accessory pathway, the tachycardia has what pattern of activation?

Answer 392

His-Ventricle-Atria

Question 393

For AVNRT, the tachycardia usually has what pattern of activation?

Answer 393

His-Atria-Ventricle... see simultaneous activation of A and V *Can be H-V-A

Question 394

For AVNRT with a His-Ventricle-Atrial pattern, where would the earliest atrial activation be?

Answer 394

Along the septum- His and CS9 (proximal CS)

Question 395

Accessory pathway-mediated SVT will have what pattern of activation?

Answer 395

H-V-A

Question 396

The earliest site of atrial activation during accessory pathway-mediated tachycardia will be where?

Answer 396

At the site where the AP is located

Question 397

A patient with D-TGA s/p atrial switch is at high risk for what type of arrhythmias?

Answer 397

Atrial arrhythmias (fibrillation, flutter, etc)

Question 398

Patients with recurrent or chronic atrial fibrillation/flutter require what?

Answer 398

Anticoagulation for thomrboprophylaxis

Question 399

How does an anti-factor Xa level work?

Answer 399

-Functional assay -Mix patients blood with known amount of active factor Xa -Depending on the level of active factor Xa after blood has been mixed (based on established nomograms), the level (and effect) of anti-factor Xa can be calculated

Question 400

An anti-factor Xa level can be used to monitor what?

Answer 400

Levels of heparin, LMWH, and direct factor Xa inhibitors (like rivaroxaban)

Question 401

What type of drug is rivaroxaban?

Answer 401

Direct factor Xa inhibitor

Question 402

What type of functional assay is a heparin test?

Answer 402

One that detects the anti-factor Xa level

Question 403

Results of a heparin test (functional assay that detects the anti-factor Xa level) will be positive for patients taking what?

Answer 403

Novel oral anticoagulants (NOACs)

Question 404

What are the 2 classes of anticoagulants commonly used in ambulatory settings?

Answer 404

-Vitamin K antagonists (warfarin) -Factor Xa inhibitors (LMWH and direct factor Xa inhibitors like edoxaban, rivaroxaban, apixaban)

Question 405

How are vitamin K antagonists administered?

Answer 405

Warfarin- PO

Question 406

True or False: Vitamin K antagonists need frequent lab monitoring and have a narrow therapeutic range?

Answer 406

True- Warfarin

Question 407

How is LMWH given?

Answer 407

Injection BID *Needs lab monitoring to maintain a safe range

Question 408

How is LMWH eliminated?

Answer 408

Renal- will need renal adjustment of dosing if creatine clearance decreases below 30mL/min

Question 409

What are some of the benefits of direct factor Xa inhibitors?

Answer 409

-Novel oral anticoagulants or direct-acting oral anticoagulants) -PO administration -Less frequent lab monitoring -Used in patients with creatinine clearance as low was 15-30mL/min without adjustment

Question 410

How are the direct factor Xa inhibitors cleared?

Answer 410

Renally- they can build up in the setting of an acute kidney injury

Question 411

What is an antiplatelet agent (platelet inhibitor) that may be used for thromboprophylaxis in patients with heart failure?

Answer 411

ASA

Question 412

True or False: At antiplatelet dosing, ASA is unlikely to cause AKI?

Answer 412

True

Question 413

Acetaminophen toxicity causes what?

Answer 413

Elevated transaminase levels

Question 414

How is enoxaparin given?

Answer 414

Injections

Question 415

What is a direct thrombin inhibitor used in patients with contraindications to heparin (like HIT)?

Answer 415

Dabigatran

Question 416

True or False: Dabigatran causes elevated anti-factor Xa levels on lab testing?

Answer 416

False

Question 417

What are 2 novel oral anticoagulants that are direct factor Xa inhibitors?

Answer 417

Apixaban Rivaroxaban

Question 418

True or False: The novel oral anticoagulants apixaban and rivaroxaban have a wide therapeutic window and don't need frequent dose titration?

Answer 418

True

Question 419

True or False: The novel oral anticoagulants apixaban and rivaroxaban will have a positive result on a heparin test or an anti-factor Xa test?

Answer 419

True

Question 420

True or False: ASA has no impact on heparin or anti-factor Xa test results?

Answer 420

True

Question 421

What should be done immediately for a patient s/p cavopulmonary anastomosis with significant hypoxia?

Answer 421

-CXR: Rule out any intrathoracic process like hemo/pneumothoraces, significant atelectasis, enlarging cardiomediastinal silhouette, or pulmonary edema -TTE: Often limited in assessing for obstruction in the cavopulmonary pathway or branch PAs, or baffle leak (for Hemi-Fontans), but should be attempted *If no cause found, do these things while arranging for cath

Question 422

For a post-operative cavopulmonary anastomosis with significant hypoxemia, what needs to be diagnosed and how once any intrathoracic or pulmonary processes are ruled out with CXR?

Answer 422

Cath- Need to assess for any post-operative complications like an obstruction in the cavopulmonary circuit or baffle leak *May need the surgeon to re-explore the patient surgically without further diagnostic testing

Question 423

True or False: The use of a transjugular Swan-Ganz catheter is not often used in PICU because of the risk of pulmonary artery perforation?

Answer 423

True- Also less likely to be diagnostic without bedside fluoroscopy and angiography

Question 424

Hypoxic patients who have undergone the bidirectional Glenn procedure or cavopulmonary anastomosis require evaluation for what?

Answer 424

-Intrathoracic processes -Pulmonary dysfunction -Cardiac dysfunction -Interrogation of the cavopulmonary circuit- Obstruction or baffle leaks

Question 425

Patients with ToF and moderate to severe _ deviation of the subpulmonary infundibulum can be prone to dynamic subpulmonary narrowing and worsening RVOTO

Answer 425

Anterior and superior

Question 426

What causes a hypercyanotic ("tet") spell?

Answer 426

Abrupt change in ratio of pulmonary to systemic blood flow

Question 427

What can precipitate a hypercyanotic spell?

Answer 427

Dehydration Agitation

Question 428

The degree of cyanosis in ToF is dictated by what?

Answer 428

-Severity of fixed and dynamic PS -Ratio of SVR to PVR *Increased subpulmonary stenosis and increased PVR promote more desaturated blood to shunt R-L across the VSD and out the aorta

Question 429

The mechanism of a hypercyanotic spell in ToF patients is often secondary to what?

Answer 429

-Changes in RV contractility -Endogenous catecholamines -Hypovolemia

Question 430

Describe the cycle of a hypercyanotic spell

Answer 430

-Changes in RV contractility, endogenous catecholamines, hypovolemia -Severe dynamic subpulmonary obstruction -Increased R-L shunting across the VSD -Leads to hypoxia and increases PVR -Leads to a vicious cycle of worsening hypoxia and increased R-L shunting of desaturated blood across the VSD

Question 431

Why is there diminished murmur intensity in a hypercyanotic spell?

Answer 431

Dynamic subpulmonary stenosis leads to decreased blood flow across the RVOT... this results in diminished murmur intensity

Question 432

What are things that can aggravate a hypercyanotic spell?

Answer 432

-Hyperpnea (often a response to acute hypoxia and secondary metabolic acidosis) -Crying -Agitation -Exertion

Question 433

True or False: Hypercyanotic spells may lead to hypoxia, syncope, seizures and even death?

Answer 433

True

Question 434

What are the clinical treatments for a hypercyanotic spell directed at?

Answer 434

Increasing the amount of pulmonary blood flow by increasing SVR and decreasing resistance to pulmonary blood flow

Question 435

What are some options for management of a hypercyanotic spell?

Answer 435

-Knees to chest (compresses femoral vessels and increases SVR) -Fluid resuscitation (increases preload) -O2 (decreases PVR and increases O2 content) -Phenylephrine (increases SVR) -Sedation (reduces O2 demands) -IV B-blocker (slow HR to decrease dynamic subpulmonary obstruction by promoting ventricular diastolic filling) *If refractory to medical therapies, may need ECMO or emergent sugery

Question 436

What is the risk of a hypercyanotic spell in ToF largely determined by?

Answer 436

Degree of anterosuperior deviation of the subpulmonary infundibulum causing subpulmonary narrowing

Question 437

What is treatment of a hypercyanotic spell directed at?

Answer 437

Increasing the amount of pulmonary blood flow by increasing SVR, reducing dynamic obstruction and decreasing PVR

Question 438

Antracycline doses above what put patients in a high-risk category for cardiotoxicity?

Answer 438

250mg/M2- although any exposure can cause myocardial injury

Question 439

Radiation exposure at levels above what places a patient at risk for developing cardiovascular side effects?

Answer 439

35Gy

Question 440

What is one of the side effects associated with radiation exposure?

Answer 440

Constrictive pericarditis

Question 441

What findings can be seen on ECG in constrictive pericarditis?

Answer 441

Low-voltage QRS

Question 442

What are some findings on echo that are consistent with constrictive pericarditis?

Answer 442

-Bright pericardial border -No significant pericardial effusion -Normal systolic biventricular function -Abnormal ventricular septal bounce -Elevated mitral e' on TDI -Mitral valve inflow variation with respiration -Hepatic vein a-wave reversal with respiration

Question 443

What findings on cath are consistent with constrictive pericarditis?

Answer 443

-Equalization of pressures in the cardiac chambers -Square-root sign appearance of the diastolic pressure tracings

Question 444

What is seen on CMR or CT in constrictive pericarditis?

Answer 444

Thickening of the pericardium

Question 445

What is a concern giving lasix to someone with constrictive pericarditis?

Answer 445

-Potential to volume deplete the patient -Reduces preload -Can result in circulatory collapse

Question 446

If PE findings are consistent with pericardial effusion in an oncology patient, but there is no pericardial effusion on echo, what diagnosis should you consider?

Answer 446

Constrictive pericarditis (especially if they received radiation)

Question 447

Why should fluids be given cautiously to someone with constrictive epricarditis?

Answer 447

-May make them symptomatic (tachypneic, etc) -Equal/elevated end diastolic and atrial pressures, can't handle more volume above a certain point

Question 448

What is definitive treatment for constrictive pericarditis?

Answer 448

Pericardiectomy or "pericardial stripping" -Removal of the thickened, fibrotic layer that develops after radiation exposure can be a definitive therapy to normalize the elevated end diastolic ventricular pressures and constrictive physiology

Question 449

What can happen to the pericardium after radiation therapy?

Answer 449

It can become thickened and fibrotic

Question 450

What is the pericardium?

Answer 450

Double-layer sac that provides lubrication for the heart

Question 451

True or False: Pericardiectomy doesn't always eliminate symptoms of constrictive pericarditis due to the difficulty completely removing an adherent pericardium

Answer 451

True

Question 452

Radiation therapy can lead to what?

Answer 452

Constrictive pericarditis *May manifest similarly to other sequelae of treatments for pediatric cancers

Question 453

What do you need to be cautious about in someone with constrictive pericarditis?

Answer 453

Fluid management... need to be cautious with fluids and diuretics

Question 454

What is the definitive therapy for constrictive pericarditis?

Answer 454

Pericardiectomy *Symptom relief is variable

Question 455

What are other names for left posterior fascicular (LPF) ventricular tachycardia?

Answer 455

-Verapamil sensitive VT -Belhassen VT

Question 456

Describe verapamil sensitive VT

Answer 456

-RBBB pattern (rsR') -LAD -Discrete Q waves in I and aVL

Question 457

What is the most common type of VT originating from the LV in young patients with a structurally normal heart?

Answer 457

Verapamil sensitive VT

Question 458

Describe patients who develop verapamil sensitive VT?

Answer 458

-Young and healthy -Often experience first episode during adolescence

Question 459

What can precipitate episodes of verapamil sensitive VT?

Answer 459

-Exercise -Excitement -Infection

Question 460

What are typical presenting symptoms of verapamil sensitive VT?

Answer 460

Palpitations Dizziness Fatigue Shortness of breath

Question 461

True or False: Verapamil sensitive VT episodes are usually well tolerated and the patients are often hemodynamically stable?

Answer 461

True

Question 462

What medication is effective for left posterior fascicular ventricular tachycardia or Belhassen tachycardia?

Answer 462

Verapamil- why called verapamil sensitive VT

Question 463

What is the typical ventricular rate in verapamil sensitive ventricular tachycardia?

Answer 463

150-220bpm

Question 464

What is verapamil sensitive ventricular tachycardia often misdiagnosed as?

Answer 464

SVT with aberrancy

Question 465

True or False: The incidence of syncope and sudden death is rare in verapamil sensitive ventricular tachycardia?

Answer 465

True

Question 466

For patients with verapamil sensitive ventricular tachycardia who have frequent episodes or are averse to long-term medical therapy, what is a good option?

Answer 466

Catheter ablation

Question 467

What is the mechanism for verapamil sensitive ventricular tachycardia?

Answer 467

-Re-entry involving the left posterior fascicle, abnormal Purkinje system and part of the adjoining myocardium in the LV with slow/decremental conduction properties -The abnormal Purkinje system is verapamil sensitive

Question 468

Where is ablation needed for verapamil sensitive ventricular tachycardia?

Answer 468

Apical third of LV septum

Question 469

True or False: Left posterior fascicular ventricular tachycardia generally doesn't respond to adenosine?

Answer 469

True (rare case reports of rate responsiveness to adenosine)

Question 470

Describe the QRS morphology in RVOT VT?

Answer 470

LBBB with inferior axis

Question 471

A rate around what is commonly seen in atrial flutter with 2:1 conduction?

Answer 471

150bpm

Question 472

If there is VA dissociation during a tachycardia, which 2 diagnoses are unlikely?

Answer 472

SVT Atrial flutter

Question 473

What are 2 medications which can be used to help with atrial flutter?

Answer 473

-Beta-blocker for rate control -Ibutilide for chemical cardioversion

Question 474

What is the most common type of VT originating in the LV in young patients with structurally normal hearts?

Answer 474

Left posterior fascicular ventricular tachycardia Belhassen VT

Question 475

What are the ECG findings consistent with left posterior fascicular ventricular tachycardia?

Answer 475

Wide-complex tachycardia with a RBBB morphology and superior axis

Question 476

Left posterior fascicular ventricular tachycardia is very responsive to what?

Answer 476

Verapamil

Question 477

For SVT, if the atrial activation occurs in the 1st half of the RR interval, it is defined as what?

Answer 477

Short RP tachycardia

Question 478

What represents the most distal electrode on a CS catheter?

Answer 478

CS1

Question 479

Where is CS1 located for an EP study?

Answer 479

Close to the left lateral MV annulus in the LAO fluoroscopic view

Question 480

The LAO view shows what view of the AV valves?

Answer 480

En face

Question 481

CS1 and CS2 during an EP study will be closest to what?

Answer 481

Left lateral MV annulus (4 o'clock position on MV annulus)

Question 482

If there is earliest atrial activation at CS1 during SVT, where is the pathway?

Answer 482

Left lateral AP

Question 483

Describe the circuit in a left lateral accessory pathway mediated tachycardia

Answer 483

-Antegrade conduction over the AV node (results in a narrow QRS complex) -Eccentric retrograde conduction over the left lateral accessory pathway (results in earliest atrial depolarization on CS1/CS2 electrode pair what is closest to the AP)

Question 484

If you have a left lateral accessory pathway, what is required for mapping and ablation?

Answer 484

A transseptal puncture to access the LA *In the past, used to use a retrograde approach from the femoral artery, but now most EP use transseptal approach

Question 485

The risk of AV block during an AP ablation is increased if the ablation target is where?

Answer 485

-Anteroseptal/parahisian -Midseptal

Question 486

Why are right-sided APs on the lateral tricuspid valve annulus more likely to recur?

Answer 486

Stability of the ablation catheter is difficult to achieve

Question 487

What is the recurrence risk of left lateral accessory pathways?

Answer 487

<5%

Question 488

Occasionally, APs in the epicardial posteroseptal and left posterior locations are associated with what?

Answer 488

Presence of a diverticulum of the CS

Question 489

If there is a diverticulum of the coronary sinus, where is it located?

Answer 489

Between the origin of the middle and posterior cardiac vein

Question 490

With an AP in the epicardial posteroseptal or left posterior location associated with a diverticulum of the CS, what may be needed to assess for the approximation to the coronary arteries to the potential ablation site in the CS diverticulum?

Answer 490

CS venogram and coronary artery angiogram *Given proximity of the coronary artery in this location, the EP may use cryoablation for these APs

Question 491

True or False: With a left lateral AP, a venogram is required prior to ablation because of the risk of damage to a coronary artery?

Answer 491

False- Left lateral MV annulus isn't the typical location of the CS diverticulum (situation where you may need a venogram due to risk for proximity to and injury of the coronary arteries... these are the APs in the epicardial posteroseptal and left posterior locations)

Question 492

What is the ablation target in accessory pathway mediated orthodromic SVT?

Answer 492

Earliest site of retrograde atrial activation

Question 493

If atrial activation falls in the first half of the RR interval, it is defined as what type of tachycardia?

Answer 493

Short RP

Question 494

What should be included in the differential diagnosis for a patient with HF symptoms and a severely enlarged cardiac silhouette on CXR in infancy?

Answer 494

-ALCAPA -Critical CoA -Critical AS -Myocarditis -Tachycardia induced cardiomyopathy -Idiopathic DCM -Metabolic abnormalities (Pompe)

Question 495

Deep Q waves in I and aVL?

Answer 495

ALCAPA

Question 496

Describe the pathophysiology of ALCAPA

Answer 496

-Based on anomalous origin of the coronary artery and normal drop in PVR over time -Initially, the LV myocardium is perfused with PA blood (lower O2 content), but relative normal perfusion pressure due to elevated PVR after birth -As PVR falls, perfusion pressure diminishes causing ischemia to LV and consequent deep Q waves in I and aVL -Eventually can result in reversal of flow into the PA via collateral arteries between the right and left coronary systems -Ischemic environment can lead to LV systolic dysfunction, infarction of the papillary muscles, significant MR and death

Question 497

How do the coronary arteries develop embryologically?

Answer 497

-Do not arise from the aortic root and grow out -Form through a process of coalescence of endothelial progenitor cells and then grow into the aortic wall (likely through the guidance of vascular endothelial growth factor and periaortic cardiomyocytes)

Question 498

What is the incidence of ALCAPA?

Answer 498

Low- 1/300,000

Question 499

When does ALCAPA present?

Answer 499

Bimodal- 90% in infancy, 10% in childhood or adulthood

Question 500

What leads to a later presentation of ALCAPA?

Answer 500

Extensive collateralization between the right and left coronary systems (may limit ischemia and heart failure) *Adolescent patients may experience sudden cardiac arrest

Question 501

How should ACLAPA be imaged on echo?

Answer 501

2D and Color Doppler in PSSA/PSLA and subcostal short axis *Imaging without color may lead to a missed ALCAPA diagnosis because the left coronary artery may be very close to the left coronary cusp

Question 502

What can be seen on echo in an infant with ALCAPA after the PVR has decreased?

Answer 502

Flow from the coronary artery into the PA

Question 503

Besides the actual coronary, what are other clues on echo to a diagnosis of ALCAPA?

Answer 503

-Echobright papillary muscles -Significant MR

Question 504

What is required if echo can't confirm a diagnosis of ALCAPA?

Answer 504

-Cath -CTA

Question 505

What should be considered in an infant with heart failure symptoms and ECG with deep Q waves in I and aVL?

Answer 505

ALCAPA

Question 506

How do the coronary arteries form?

Answer 506

-Process of coalescence of endothelial progentior cells -Grow into the aortic wall

Question 507

Echo imaging of the left coronary artery without color Doppler may lead to a missed diagnosis of what?

Answer 507

ALCAPA

Question 508

What is a lysosomal transport disorder classified as a glycogen storage disease (type IIb) without the metabolic components classically associated with glycogen storage diseases?

Answer 508

Danon disease

Question 509

What causes Danon disease?

Answer 509

Mutation in the lysosomal-associated membrane protein-2 (LAMP-2) *Impairs the process of transporting cellular material into the lysosome

Question 510

Which gender is more significantly affected by Danon disease?

Answer 510

Males (childhood or adolesnce) and more severely than females

Question 511

Describe the cardiac findings seen in Danon disease?

Answer 511

-Cardiomyopathy (most often HCM, but DCM has bee described) -WPW/pre-excitation -Pseudo-preexcitation -Risk for arrhythmia

Question 512

Describe the non-cardiac findings seen in Danon disease?

Answer 512

-Proximal skeletal myoathy: Upper arms, shoulders, neck, upper thighs -Possible elevated creatine kinase levels -Most (but not all) male patients have mild intellectual disability (can be overlooked)

Question 513

Describe the presentation of Danon disease in a female?

Answer 513

-Affected in early adulthood -Develop cardiomyopathy (50% HCM, 50% DCM) -Less likely to have skeletal myopathy or intellectual disabilities

Question 514

True or False: Children with Danon disease can get a heart transplant?

Answer 514

True *Some case reports suggest that steroids may exacerbate skeletal myopathy

Question 515

What is a progressive X-linked lysosomal disorder of glycosphingolipid metabolism as a result a deficient or absent lysosomal a-galactosidase A activity?

Answer 515

Fabry disease

Question 516

What is another name for Fabry disease?

Answer 516

Anderson-Fabry disease

Question 517

What are some of the symptoms in childhood associated with Fabry disease?

Answer 517

-Pain: Episodic crises with agonizing, burning pain that originates in the extremities and radiates inwards and chronic pain with burning or tingling paresthesia triggered by or accompanied by fever -GI symptoms -Anhidrosis/hypohidrosis -Skin lesions -Eye exam findings -Kidney involvement -Cardiac disease

Question 518

What are the cardiac manifestations of Fabry disease?

Answer 518

-Presents in adolescence -LVH -Shortened PR interval -Arrhythmias -Valvular insufficiency *With age, progressive myocardial fibrosis that leads to systolic dysfunction and malignant arrhythmias

Question 519

Which gender manifests symptoms earlier and in a more severe form with Fabry dsiease?

Answer 519

Males (as compared to female heterozygotes)

Question 520

True or False: There is a cardiac variant of Fabry disease without overt systemic involvement that has been shown to account for up to 4% of cases of HCM?

Answer 520

True

Question 521

What can be done to halt disease progression in Fabry disease?

Answer 521

Enzyme replacement therapy

Question 522

True or False: Fabry disease is associated with intellectual disabilities?

Answer 522

True

Question 523

What is an AR disorder that prevents the body from converting certain fats to energy during periods of stress?

Answer 523

Long-chain L-3 hydroxyacyl-CoA dehydrogenase deficiency (LCHAD)

Question 524

What mutation causes LCHAD?

Answer 524

HADHA gene- Affects production of an enzyme complex (mitochondrial trifunctional protein)

Question 525

What are the signs and symptoms of LCHAD?

Answer 525

-Appear during infancy or early childhood -Feeding difficulties -Lethargy -Hypoglycemia -Hypotonia -Liver problems -Eye problems -Cardiomyopathy

Question 526

What is important in the management of LCHAD?

Answer 526

Early recognition... dietary change can be beneficial

Question 527

True or False: LCHAD would be in the differential of a late-onset cardiomyopathy?

Answer 527

False

Question 528

What is a glycogen storage disease with abnormal production and function of the acid alpha-glucosidase enzyme?

Answer 528

Pompe disease

Question 529

A mutation in what causes Pompe?

Answer 529

GAA gene

Question 530

What are the 3 forms of Pompe?

Answer 530

-Classic infantile onset -Non-classic infantile onset -Late-onset

Question 531

Describe classic infantile onset Pompe

Answer 531

-Begins within first 3 months of life -Muscle weakness -Hypotonia -Hepatomegaly -HCM

Question 532

Describe the non-classic infantile onset Pompe?

Answer 532

-Appears by age 1 -Delayed motor skills -Progressive muscle weakness -May have HCM, but less likely to present with heart failure

Question 533

Describe late-onset Pompe

Answer 533

-May present in adolescence or adulthood with progressive muscle weakness in legs and trunk -May have CV abnormalities on baseline ECG or Echo (including pre-excitation), but unlikely to have significant hypertrophy as classically seen in infantile Pompe

Question 534

What is a lysosomal transport disorder secondary to a mutation in the LAMP2 gene that causes HCM in young males and HCM/DCM in adult females?

Answer 534

Danon

Question 535

Danon, Fabry and Pompe disease can have what findings on ECG?

Answer 535

-Pre-excitation or WPW -LVH -Short PR interval

Question 536

True or False: Late-onset cardiomyopathy can be associated with metabolic disorders?

Answer 536

True *Metabolic diseases are in the differential for infantile cardiomyopathies

Question 537

What is one of the more rare causes of myocarditis?

Answer 537

Giant cell myocarditis

Question 538

True or False: Giant cell myocarditis is only seen in adults?

Answer 538

False- can be seen in children and adults

Question 539

True or False: Giant cell myocarditis can be refractory to treatment in many cases?

Answer 539

True

Question 540

What problem is giant cell myocarditis associated with?

Answer 540

Development of complete heart block (5%)

Question 541

What is seen on histology for giant cell myocarditis?

Answer 541

-Mixed infiltrate of mononuclear cells, eosinophils, histiocytes, and multinucleated giant cells with extensive myocyte necrosis

Question 542

In terms of function and dilation, what is seen in fulminant myocarditis?

Answer 542

Depressed biventricular function without ventricular dilation (indicative of an acute process)

Question 543

True or False: Patients with giant cell myocarditis often have acute fulminant myocarditis and may need intensive care, including PPV?

Answer 543

True

Question 544

There is an association between giant cell myocarditis and what?

Answer 544

Immune disorders

Question 545

Giant cell myocarditis is in a group of causes of myocarditis that response to what?

Answer 545

Immunosuppression (especially steroid-based regimens)

Question 546

True or False: Patients who go on to develop chronic heart failure symptoms after myocarditis may need to be listed for heart transplant?

Answer 546

True

Question 547

True or False: Treating a patients with immunomodulatory and immunosuppressive therapies may treat the inflammatory process and help with myocardial recovery in giant cell myocarditis?

Answer 547

True- try this before listing for transplant

Question 548

Why are transplant outcomes for patients with giant cell myocarditis less favorable than for those with other forms of cardiomyopathy?

Answer 548

-Giant cell infiltration of the donor heart has been reported *Heart transplant is still a potential therapeutic option

Question 549

Compared with viral myocarditis, there is a wider consensus to use what in giant cell myocarditis?

Answer 549

Combination immunomodulatory therapeutics

Question 550

What has been shown to ameliorate the effects of giant cell myocarditis such that it can potentially go into clinical remission and allow for recovery from heart failure in approximately 2/3 of those affected?

Answer 550

Corticosteroids + Immunosuppressants (cyclosporine or azathioprine)

Question 551

What medication is used in recurrent or refractory cases of giant cell myocarditis?

Answer 551

Rituximab- not a first-line agent for the treatment of myocarditis (GCM or any other type)

Question 552

What is rituximab?

Answer 552

Monoclonal antibody therapy directed to the CD20 antigens on B-cells *Used in recurrent or refractory cases of giant cell myocarditis

Question 553

What is anakinra?

Answer 553

IL-1 receptor antagonist

Question 554

When is anakinra used in myocarditis?

Answer 554

For atypical or refractory cases- not considered 1st line therapy

Question 555

True or False: Giant cell myocarditis represents a rare etiology for myocarditis and one that is associated with a poor prgonsis?

Answer 555

True

Question 556

What is often used as 1st line therapy for giant cell myocarditis?

Answer 556

Immunomodulatory and immunosuppressive agents (differentiates from more routine viral myocarditis

Question 557

What are some of the etiologies of myocarditis?

Answer 557

-Viral -Inflammatory -Toxin -Drug

Question 558

Describe an outflow tract PVC

Answer 558

-PVC axis positive in inferior leads (focus from more superior portion of heart- outflow) -LBBB morphology with transition at V3-V4 (suggests coming from RVOT)

Question 559

What does a transition earlier than V3-V4 suggest as origin of a PVC with LBBB morphology?

Answer 559

Coronary cusps

Question 560

True or False: PVCs can be frequently encountered in pediatric patients and tend to have a good overall prognosis in asymptomatic patients who have uniform PVCs and a structurally normal heart with no concerning family history?

Answer 560

True

Question 561

True or False: It is unlikely for patients with uniform PVCs and no cardiac abnormalities to have worsening of their clinical status over time?

Answer 561

True

Question 562

What is usually required in patients with PVCs?

Answer 562

Observation

Question 563

A PVC burden over what % on a 24 hour Holter monitor increases the risk of developing ventricular dysfunction and warrants longitudinal observation?

Answer 563

10-20%

Question 564

What needs to be considered as an underlying diagnosis in a patients with PVCs that arise from the RVOT?

Answer 564

ARVC- PVCs could be an early manifestation

Question 565

If a patient is having >500 ectopic beats in a 24 hour period that are RVOT in origin and a concerning personal/family history or echo findings suggestive of ARVC, what should be considered?

Answer 565

CMR

Question 566

Medical or catheter treatments in patients with frequent PVCs is usually reserved for who?

Answer 566

-Symptomatic -Ventricular dysfunction -Hemodynamically significant ventricular arrhythmias

Question 567

What are the most common used first line medications for PVCs that warrant treatment?

Answer 567

-B-blockers -CCB (if >1 year)

Question 568

True or False: Outflow tract PVCs overall tend to have a benign course?

Answer 568

True

Question 569

What is considered mild-moderate hypertriglyceridemia?

Answer 569

150-500

Question 570

What are some things that can cause mild-moderate hypertriglyceridemia?

Answer 570

-Obesity -Type 2DM -Thyroid dysfunction -Renal or hepatic disease -Drugs: PO retinoids, thiazides, B-blockers, estrogens, glucocorticoids, HIV medications (protease inhibitors), antipsychotics -Alcohol

Question 571

True or False: Lifestyle changes will often normalize triglyceride levels in most patients with mild-moderate elevation?

Answer 571

True

Question 572

What is considered severe elevation in triglyceride levels?

Answer 572

>1000

Question 573

Severe elevations in TG over 1000 is a clue to what?

Answer 573

Underlying monogenic or polygenic inheritance

Question 574

A small subset of patients with severe hypertriglyceridemia have what?

Answer 574

Familial chylomicronemia syndrome

Question 575

What is the breakdown of inheritance types in familial chylomicronemia syndrome?

Answer 575

80% AR 20% genetic defects in APOC2, APOA5, LMF1, GP1HBP1

Question 576

What causes AR familial chylomicronemia?

Answer 576

Genetically mediated deficiency of lipoprotein lipase (LPL) enzyme resulting in poor catabolism of triglyceride-rick lipoproteins such as chylomicrons

Question 577

Genetic defects in APOC2, APOA5, LMF1, GP1HBP1 cause what?

Answer 577

Familial chylomirconemia syndrome- these genes encode cofactors or binding proteins required for efficient functioning of the LPL enzyme

Question 578

How do patients with familial chylomicronemia syndrome present?

Answer 578

Childhood or adolescence with recurrent bouts of acute pancreatitis secondary to clogging of the pancreatic capillaries with TG-rich plasma

Question 579

What is seen on physical exam in familial chylomirconemia?

Answer 579

-TG-laden yellowish papules on the extremities, torso and back (eruptive xanthomas) -Lipemia retinalis -Hepatosplenomegaly

Question 580

What is the hallmark of familial chylomicronemia?

Answer 580

Milky/turbid plasma due to excess chylomicrons

Question 581

What is seen on lipid profile in familial chylomicronemia?

Answer 581

-Serum triglyceride levels are markedly elevated -Normal to subnormal levels of remaining lipoproteins

Question 582

How is familial chylomicronemia diagnosed?

Answer 582

-Clinical history -Lipid profile (elevated TG levels, normal to subnormal levels of remaining lipoproteins) *Genetic testing can be done for a definitive diagnosis, but isn't widely available

Question 583

What is the treatment for familial chylomicronemia?

Answer 583

-Restricting fat intake to <10-15% caloric intake (15-20g/day) -Avoid alcohol -Limit simple, refined carbohydrates *Despite strict adherence, patients will have breakthrough episodes of pancreatitis

Question 584

In familial chylomicronemia, what is needed in terms of dietary considerations?

Answer 584

-Restricting fat intake to <10-15% caloric intake (15-20g/day) -Avoid alcohol -Limit simple, refined carbohydrates -Work with RD/nutritionist -Monitor levels of fat-soluble vitamins -Consider MCT oil (metabolized via a chylomicron independent pathway)

Question 585

What is used in mild-moderate hypertriglyceridemia, but is unlikely to be beneficial in patients with familial chylomicronemia?

Answer 585

Fibrates and omega-3 fatty acids

Question 586

What is Volanesorsen?

Answer 586

Antisense oligonucleotide inhibitor (ASO) of apo C3 which reduces rates of pancreatitis and TG levels in familial chylomicronemia *Conditionally approved in Europe *Limited by high cost

Question 587

True or False: There are currently no medications approved by the FDA to treat familial chylomicronemia?

Answer 587

True

Question 588

What are the first-line agents to treat familial hypercholesterolemia?

Answer 588

Statins

Question 589

What is the MOA of a statin?

Answer 589

Inhibition of the 3-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase) -Rate limiting enzyme in cholesterol biosynthesis -Upregulates LDL receptors in the liver

Question 590

Besides lowering LDL, what else do statins do?

Answer 590

-Modest effect on lowering TG levels -Raise HDL levels

Question 591

What is the expected effect on the LDL cholesterol lowering with a statin dependent on?

Answer 591

-Dose -Intensity

Question 592

What is the mainstay of treatment in familial chylomicronemia?

Answer 592

-Fat restricted diet -Can use statin or fibrate as an adjunct to lower TG levels

Question 593

What is an AR condition characterized by severe hypertriglyceridemia with serum TG levels >1000?

Answer 593

Familial chylomicronemia

Question 594

Patients with familial chylomicronemia have a genetically mediated deficiency of what?

Answer 594

Lipoprotein lipase

Question 595

What is recommended to prevent pancreatitis and is the mainstay of therapy for familial chylomicronemia?

Answer 595

-Strict adherence to a diet regimen limiting fat intake to <10-15% of total caloric intake (15-20g/day) -Avoidance of alcohol -Limiting simple, refined carbohydrates

Question 596

What are the effects of NSAIDs on PGE?

Answer 596

NSAIDs reduce the production of PGE2- this is necessary to maintain patency of the fetal ductus arteriosus

Question 597

MOA of NSAIDs?

Answer 597

Cyclooxygenase inhibitor

Question 598

Where does the fetal ductus arteriosus connect?

Answer 598

From the PA to the descending aorta

Question 599

Describe the purpose of the fetal ductus arteriosus?

Answer 599

-Lack of gas exchange in the lungs in utero leads to a consequently elevated PVR -Ductus arteriosus provides egress from the right heart into the systemic circulation (where RV output can be sent to placenta for gas exchange)

Question 600

What % of the combined cardiac output is ejected by the RV?

Answer 600

60% -Majority is directed through the ductus arteriosus and a small volume goes to the branch PAs with that volume increasing over gestation

Question 601

True or False: Restriction of the ductus arteriosus in utero can have serious and potentially deadly hemodynamic consequencyes?

Answer 601

True

Question 602

True or False: Constriction of the ductus arteriosus is likely more common than complete closure?

Answer 602

True

Question 603

What causes constriction or complete closure of the ductus arteriosus?

Answer 603

-Extrinsic agents that have been ingested by Mom -Most well-known are NSAIDs (ibuprofen, indomethacin, ASA) -Less well known: Sympathomimetic drugs like pseudoephedrine, yerba mate tea, various herbal remedies *Sometimes etiology is unknown

Question 604

What are the effects of constriction or complete premature closure of the ductus arteriosus?

Answer 604

-Increased afterload to the RV -Results in dilation and hypertrophy of the RV and TR -Most concerning- these changes can result in right sided ventricular systolic dysfunction and right sided heart failure leading to hydrops and fetal death -Increased volume delivered to PAs can cause muscularization of pulmonary vasculature with long-term elevation of PVR and PPHN -Changes can reduce volume of blood ejected via RV and increase in R-L atrial shunting (In some patients, this can cause RV hypoplasia and PS or PA)

Question 605

What is seen on fetal echo in ductal construction?

Answer 605

-Dilated RV -TR -Narrowed ductus arteriosus with increased systolic/diastolic velocities and Doppler pattern similar to what is seen in CoA

Question 606

What should be done for fetal patients with ductal constriction?

Answer 606

-Detailed history to identify causative agent and stop its ingestion -Monitoring for hydrops -Consider early delivery- will decrease PVR and relieve afterload on right heart

Question 607

How is postnatal diagnosis of ductal constriction suggested?

Answer 607

No visualization of a ductus arteriosus in the 1st 24 hours in the above clinical settings

Question 608

True or False: Lamotrigine is a new antiepileptic drug that has a low teratogenicity profile?

Answer 608

True

Question 609

True or False: In general, antiepileptic drugs are throught to increase risk for major birth defects, but the specific increased risk for CHD is low?

Answer 609

True

Question 610

Fetal exposure to ACEi (like lisinopril) in the 2nd and 3rd trimester is associated with what?

Answer 610

Fetal renal failure, oligohydramnios and death

Question 611

What does prenatal exposure to lithium increase the risk of?

Answer 611

Right heart disease, specifically Ebstein anomaly of the tricuspid valve

Question 612

Constriction of the fetal ductus arteriosus is most often caused by what?

Answer 612

Ingestion of an extrinsic agent such as an NSAID (ibuprofen or indomethacin), pseudoephedrine and herbal agents

Question 613

Fetal ductal arteriosus constriction can result in what prenatal problems?

Answer 613

-TR -RV dilation and failure -Hydrops -Fetal death

Question 614

What are the postnatal effects of a constricted fetal ductus arteriosus due to?

Answer 614

Muscularization of the pulmonary vascular bed resulting in persistently elevated PVR

Question 615

What is the differential diagnosis for a narrow complex short RP tachycardia?

Answer 615

-SVT (AVRT or typical AVNRT) -Junctional tachycardia

Question 616

If there is a tachycardia in a neonate that terminates abruptly with vagal maneuvers, what type is most likely to be?

Answer 616

SVT

Question 617

In infants, what is the most common mechanism of SVT?

Answer 617

AVRT caused by an accessory pathway (concealed or manifest)

Question 618

True or False: AVNRT is less commonly the cause of SVT in an infant?

Answer 618

True

Question 619

True or False: Junctional tachycardia is unlikely to convert with vagal maneuvers?

Answer 619

True

Question 620

In cases of junctional tachycardia, what is true about the arrhythmia?

Answer 620

Tends to be recalcitrant

Question 621

Why is there an increased concern that infants might not receive care for SVT until they have developed cardiac dysfunction?

Answer 621

They can't communicate when they are having SVT *Can have a higher morbidity and mortality compared with older children and adults with SVT

Question 622

What is the consensus among pediatric cardiologists for the management of SVT in infants?

Answer 622

Medically treat infants with documented SVT to prevent arrhythmia recurrence

Question 623

What are the most common antiarrhythmics used in infants with SVT?

Answer 623

-B-Blockers -Digoxin (in absence of preexcitation)

Question 624

Besides beta blockers and digoxin, what medication can also be considered as a first-line agent for older patients with SVT?

Answer 624

CCB

Question 625

When is amiodarone used in the management of SVT?

Answer 625

As a 2nd or 3rd line agent... carries a significant potential for adverse effects

Question 626

Why is an EP study and ablation not a first line option in infants with SVT?

Answer 626

Most infants with SVT will have spontaneous resolution of their tachycardia during their first year after birth

Question 627

Which infants with SVT be require an ablation to be considered?

Answer 627

Those with recalcitrant SVT that fails multiple drug management and is hemodynamically significant

Question 628

The risks of ablation are significantly higher in patients who weight < what?

Answer 628

15kg

Question 629

What are some of the risks with ablation in a patient <15kg?

Answer 629

-Perforation -AV node injury

Question 630

What are causes of a short RP tachycardia?

Answer 630

-AVRT -Typical AVNRT

Question 631

What are the most common first-line agents for the management of SVT in infants?

Answer 631

-B-blockers -Digoxin (if no preexcitation)

Question 632

EP studies and ablation can be considered as 1st line treatment for SVT once patients weight > what?

Answer 632

15kg

Question 633

True or False: Cardiac abnormalities in Turner syndrome (XO and mosaic variants) are common affecting more than half of all girls and women with Turner syndrome?

Answer 633

True

Question 634

When is echo done for patients with Turner's?

Answer 634

At time of diagnosis (usually in childhood)

Question 635

What things might echo miss for a patients with Turner's?

Answer 635

-PAPVR -Aortic dilation or elongation

Question 636

What testing facilitates anatomical and physiologic assessment of cardiothoracic structures that may be abnormal in girls with Turner syndrome, but missed on screening echo?

Answer 636

MRI/MRA (no ionizing radiation

Question 637

Besides echo, what imaging is recommended for girls and young women with Turner syndrome to under?

Answer 637

Cardiac MRI once they reach the age they will no longer require sedation

Question 638

PAPVR has been reported in what % of girls with Tuner syndrome?

Answer 638

3-16% *But because it is rare otherwise, the RR of PAPVR in Turner v. the normal population is high (300:1)

Question 639

Besides visualization of PAPVR, what are some other signs by MRI that there is PAPVR?

Answer 639

-Increased flows in the low SVC v. high SVC -Increased flow across the TV compared to MV -Increased flow across the PV compared with the AV -Increased Qp:Qs -RV SV that is twice the LV SV -RV dilation

Question 640

An ASD is seen in what % of girls with Turner syndrome?

Answer 640

2-5%

Question 641

In an isolated PDA, is there higher flow across the MV v. TV on MRI?

Answer 641

Mitral

Question 642

In an isolated PDA, is there higher flow across the AV or PV?

Answer 642

AV

Question 643

In a VSD, is there higher flow across the MV v. TV on MRI?

Answer 643

Mitral

Question 644

In a VSD, is there higher flow across the AV or PV?

Answer 644

PV

Question 645

In young teens with Turner syndrome, what is recommended to assess for occult CHD like pulmonary venous anomalies and aortic dilation, even in those previously reported to have had normal echo findings?

Answer 645

MRI

Question 646

True or False: CMR with phase-contrast flow techniques can be used to quantify cardiac shunt without the need for cardiac cath?

Answer 646

True

Question 647

What are the most prevalent types of CHD in Turners?

Answer 647

-Bicuspid AoV -CoA -PAPVR -VSD -ASD

Question 648

Describe the location of the conduction system in Ebstein

Answer 648

-Normal conduction system had normal location and structure of the AV node, bundle of His and LBBB -Compact AV node may be displaced towards the coronary sinus os in the Koch triangle

Question 649

Most patients with Ebstein hearts will have what on their ECG in the absence of preexcitation?

Answer 649

RBBB (71-94%)

Question 650

What causes the RBBB seen on many Ebstein patients ECGs?

Answer 650

-Multiple congenital abnormalities of the RBB (atresia, short length, narrow caliber, fibrosis) -Electrically abnormal tissue of the atrialized RV

Question 651

Besides RBBB, what else can be seen on ECG in Ebstein patients in the absence of preexcitation?

Answer 651

First-degree AB block

Question 652

What causes first degree AV block in Ebstein patients?

Answer 652

Long interatrial conduction time secondary to RA enlargement

Question 653

Patients with Ebstein anomaly are predisposed to what?

Answer 653

-Supraventricular and ventricular arrhythmias -Risk of SVD

Question 654

What are the most common type of arrhythmias in Ebstein patients?

Answer 654

Supraventricular due to AP pathways (10-38%)

Question 655

Almost all AP in Ebstein patients are on what side?

Answer 655

Right

Question 656

True or False: Nearly half of the patients with Ebstein anomaly with AP have multiple APs

Answer 656

True

Question 657

More than 75% of the APs in Ebstein anomaly are capable of what?

Answer 657

Antegrade conduction- Manifest as preexcitation on ECG (suggestive of WPW)

Question 658

What happens in a patient with Ebstein who has a right sided AP that fuses with an underlying RBBB?

Answer 658

QRS complex can become pseudonormalized, preexcitation may be subtle and RBBB may not be as evidence

Question 659

Absence of RBBB in a patient with Ebstein anomaly should raise suspicion for what?

Answer 659

Preexcitation

Question 660

Successful ablation of a right sided accessory pathway with antegrade conduction should show what?

Answer 660

Underlying RBBB

Question 661

True or False: Catheter ablation of accessory pathways in patients with Ebstein anomaly is challenging with recurrence rates as high as 20-40%

Answer 661

True

Answer 662

-Fractionated ow amplitude ventricular ECGs from the atrialized RV -Presence of multiple APs -Dilated RA -Difficulty identifying the true AV groove making catheter navigation difficult (this is thin which increases risk of RCA injury and may also have ridge tissue)

Question 663

Besides AP-mediated SVT (orthodromic and antidromic), Ebstein patients may also have SVT mediated by what?

Answer 663

-Atriofascicular (Mahaim) fibers (5%) -AVNRT (8-13%)

Question 664

What is a potential substrate for ventricular tachycardia in Ebstein patients?

Answer 664

Abnormal atrialized RV tissue

Question 665

Besides the intrinsic rhythm abnormalities in Ebstein patients, they are also prone to develop what after surgery?

Answer 665

Atrial macro reentry tachycardia, focal atrial tachycardia, atrial fibrillation, polymorphic VT

Question 666

Most patients with Ebstein anomaly have what on their baseline ECG in the absence of preexcitation?

Answer 666

RBBB

Question 667

Of all the CHD, what has the highest incidence of APs

Answer 667

Ebstein

Question 668

75% of APs in Ebstein are what type?

Answer 668

Manifest (WPW) and almost always right sided

Question 669

The absence of RBBB in patients with an Ebstein anomaly should raise suspicion for what?

Answer 669

Preexcitation

Question 670

A successful ablation of a right-sided AP with antegrade conduction in a patient with Ebstein exposes what?

Answer 670

Underlying RBBB

Question 671

What are the differences between a true positive, true negative, false positive and false negative?

Answer 671

-True Positive: Tests positive, has disease -True Negative: Tests negative, doesn’t have disease -False Positive: Tests positive, doesn’t have disease -False Negative: Tests negative, has disease

Question 672

What is the proportion of patients who truly have the disease who test positive?

Answer 672

Sensitivity

Question 673

What does sensitivity represent?

Answer 673

Chance of accurately ruling in a disease using the test

Question 674

How is sensitivity calculated?

Answer 674

Proportion of true disease + patients (T+ and F-) that have a positive result on the screening test

Question 675

What is the equation for sensitivity?

Answer 675

TP/(TP+FN) * 100

Question 676

What is the proportion of subjects who truly don’t have the disease who test negative?

Answer 676

Specificity

Question 677

What does specificity represent?

Answer 677

Chance of accurately ruling out a disease using a negative result on a screening test

Question 678

How is specificity calculated?

Answer 678

Proportion of true disease-negative patients (true negatives and false positives) that have a negative result on the screening test)

Question 679

What is the equation for specificity?

Answer 679

TN/(TN + FP) * 100

Question 680

What is the chance of a patient with a positive result truly having disease?

Answer 680

PPV

Question 681

What is the equation for PPV?

Answer 681

TP/(TP + FP) * 100

Question 682

What is the chance of a patient with a negative result truly not having the disease?

Answer 682

NPV

Question 683

What is the equation for NPV?

Answer 683

TN/(TN + FN) * 100

Question 684

In a 2x2 table, what goes on the X and Y axis?

Answer 684

-X: Disease +/- -Y: Test +/-

Question 685

In an umbilical artery Doppler on fetal echo, what does systolic pulsatile flow above the baseline represent?

Answer 685

Fetal HR

Question 686

An estimated _% of CO is sent to the placenta for gas exchange?

Answer 686

30

Question 687

True or False: A UA Doppler in which diastolic flow briefly reverses is normal?

Answer 687

False

Question 688

What type of resistor is a healthy placenta?

Answer 688

Low- should easily receive fetal blood flow (continuous antegrade flow in the UA)

Question 689

What should be seen on UA Doppler?

Answer 689

Continuous antegrade flow

Question 690

What accounts for the normal low fetal SVR?

Answer 690

Placental being a low resistor and easily receiving fetal blood flow

Question 691

What happens to the SVR after birth?

Answer 691

Arises abruptly once umbilical cord is clamped

Question 692

Absent or reversed end diastolic flow velocity in the umbilical artery is a sign of what?

Answer 692

Increased impedance in the placenta vascular bed

Question 693

Absent or reversed end diastolic flow velocity in the umbilical artery is a sign of what?

Answer 693

Placental insufficiency related to vascular maldevelopment

Question 694

True or False: Absent or reversed end diastolic flow velocity in the UA is associated with an increased risk of perinatal mortality?

Answer 694

True

Question 695

Absent or reversed end-diastolic flow velocity is a frequent finding in what?

Answer 695

Fetal growth restriction- This has important implications for pregnancy management including consideration and timing of premature delivery

Question 696

True or False: Abnormal UA Doppler has increased prevalence in fetal chromosomal abnormalities and some congenital malformations?

Answer 696

True

Question 697

If a fetus is measuring <10th percentile, what should you consider?

Answer 697

Fetal growth restriction

Question 698

True or False: Noninvasive prenatal testing (NIPT) has a high sensitivity for aneuploidies (T21, T13, T18)

Answer 698

True- A negative screen is reassuring regarding these abnormalities

Question 699

What has been described on fetal echo in severe Ebstein anomaly/tricuspid valve dysplasia and fetal complete heart block?

Answer 699

-Abnormalities in UA Doppler- physiological explanations include circular shunt physiology (Ebstein) and prolongation of the diastolic phase of the cardiac cycle (heart block) -Presence of abnormal UA Doppler is associated with poor outcome

Question 700

What extracardiac Doppler assessments are important for the evaluation of fetal hemodynamics?

Answer 700

-UA -UV -Ductus venosus -MCA -Abnormal patterns alert to derangements in the fetal circulation that may be of diagnostic and prognostic significance

Question 701

UA Doppler patterns provide insight into what?

Answer 701

Fetal circulation and well-being

Question 702

Describe a normal UA Doppler

Answer 702

-Systolic pulsatile upstrokes -Continuous antegrade diastolic flow

Question 703

Absent end-diastolic or reversed end-diastolic flow velocity in the UA are consistent with what?

Answer 703

Placental insufficiency (predict adverse perinatal outcomes)

Question 704

What are abnormal features of a ductus venosus Doppler?

Answer 704

A wave absence or reversal

Question 705

What are abnormal features of an umbilical vein Doppler?

Answer 705

-UV pulsations -Lower velocity

Question 706

What are abnormal features of an umbilical artery Doppler?

Answer 706

-Absent/reversed end diastolic flow -Elevated pulsatility index

Question 707

What is the relationship of the aorta and pulmonary artery in d-TGA?

Answer 707

-Aorta is anterior and rightward -Pulmonary artery is posterior and leftward

Question 708

What defines TGA?

Answer 708

Connection of the great arteries to their respective ventricles (not solely on the arrangement of the great arteries in space)

Question 709

What is it called when the connections of the great arteries result in the systemic and pulmonary circulations being in parallel rather than in series?

Answer 709

d-TGA

Question 710

Patients with d-TGA are dependent on what for survival?

Answer 710

-Mixing and shunting (either at atrial or ventricular level) -Shunting (through PDA or bronchial collaterals)

Question 711

Besides PGE and possible BAS, what should also be considered for newborns with d-TGA and cardiorespiratory impairment or severe hypoxemia in babies with d-TGA and IVS?

Answer 711

-Intubation -Correction of metabolic derangements and acidosis -Administer O2

Question 712

What is the benefit of PGE in d-TGA?

Answer 712

Keep the PDA open to promote volume loading of the LA and atrial level mixing

Question 713

What is a good starting dose for PGE in d-TGA?

Answer 713

-0.0125ug/kg/min -Low dose allows for ducal patency, but minimizing the risk of PGE associated apnea and need for intubation -Higher doses may be needed if PDA is small

Question 714

Why is a PDA bidirectional in the newborn period?

Answer 714

High PVR

Question 715

In a patient with d-TGA, what might need done to ensure proper mixing?

Answer 715

BAS (especially if there is a restrictive atrial level communication)

Question 716

True or False: For infants with d-TGA, performing a BAS will allow the PGE to be stopped

Answer 716

True, but for some infants even if the BAS is technically effective, they may still require PGE to improve effective pulmonary blood flow

Question 717

True or False: In patients with d-TGA and VSD, adequate mixing may not occur at the ventricular level and a BAS may still be required?

Answer 717

True

Question 718

What characterizes d-TGA?

Answer 718

Parallel circulation (patients are dependent on adequate mixing for survival

Question 719

What is needed soon after diagnosis in order for adequate mixing to occur in patients with d-TGA with a restrictive atrial level communication?

Answer 719

BAS

Question 720

What is the most common cardiomyopathy seen in children?

Answer 720

DCM

Question 721

What is characterized by left ventricular dilation and systolic dysfunction?

Answer 721

DCM

Question 722

How does DCM present?

Answer 722

Varies widely: Some patients are asymptomatic (detected after screening due to family history), other may be in acute decompensated heart failure

Question 723

What are the two ways to classify patients in acute heart failure based on symptoms?

Answer 723

-Congestion (wet or dry) -Adequacy of perfusion (warm or old)

Question 724

What is the classification of a patient presenting in acute decompensated heart failure who has elevated atrial pressure (high CVP), tachypnea, hepatomegaly, CXR with cardiomegaly, pulmonary edema, pleural effusions, hypotension and cool extremities?

Answer 724

Wet and cold

Question 725

What are the overall goals of management for someone presenting in “wet and cold” acute decompensated heart failure?

Answer 725

-Symptomatic relief -Prevention of end-organ dysfunction (optimize fluid status and CO)

Question 726

What is important therapy to reduce right and left ventricular filling pressure in the setting of pulmonary edema?

Answer 726

Diuresis

Question 727

Under what circumstance do you need to use diuretics cautiously in a patient with heart failure?

Answer 727

Concomitant low CO- diuretics have the potential to reduce preload and worsen end-organ perfusion

Question 728

Besides diuretics, what can be done for patients with respiratory symptoms from pulmonary edema?

Answer 728

-O2 -Noninvasive or invasive PPV

Question 729

How can mechanical ventilatory support help a patient in decompensated heart failure?

Answer 729

-Decrease work of breathing -Decrease LV afterload

Question 730

What is a risk of mechanical ventilatory support in patients with acute decompensated heart failure?

Answer 730

Significant hemodynamic instability with the sedation needed to intubate

Question 731

What medications have a clear role in the stabilization of patients with circulatory compromise, but should be used with caution given proarrhythmic effects?

Answer 731

Inotropes

Question 732

What medications result in a reduction in the rate and force of myocardial contraction, heart and BP and are thus contradicted in the setting of acute decompensated heart failure?

Answer 732

B-blockers

Question 733

When should B-blockers be used in heart failure?

Answer 733

Chronic management, they are contradicted in the setting of acute decompensated heart failure

Question 734

Why is neurohumoral modulation an important tenet of therapy for chronic heart failure?

Answer 734

There is activation of the sympathetic nervous system ad upregulation of the renin-angiotensin-aldosterone system (RAAS) that results in myocardial dysfunction and adverse cardiac remodeling

Question 735

Once acute symptomatic relief and adequate CO are achieved for patients in acute heart failure, what should be initiated as part of chronic management for neurohumoral modulation of the RAAS?

Answer 735

ACEi or ARB

Question 736

What medication is used in chronic heart failure management to mitigate the effects of sympathetic nervous system activation in heart failure?

Answer 736

B-blocker

Question 737

What type of B-blockers have a relative selectivity for B1 adrenergic receptors found in the heart?

Answer 737

2nd generation- metoprolol

Question 738

What type of B-blockers are nonselective for B-receptors with additional a-adrenergic blocking activity that results in cardiac reverse remodeling effects?

Answer 738

3rd generation- carvedilol

Question 739

What medication used in chronic heart failure therapy has the ability to oppose adrenergic stimulation at multiple sites?

Answer 739

Carvedilol

Question 740

What are adverse effects from B-blockers in pediatric patients with heart failure?

Answer 740

Fatigue, hypotension, dizziness, headaches, depression, mood disorder

Question 741

What are some contraindications to B-blockers?

Answer 741

Severe bradycardia, high-grade heart block, hypotension, acute decompensated heart failure, bronchospasm/asthma history (relative contraindication- 2nd generation agents with a relative selectivity for B1-adrenergic receptors may be cautiously considered when the history of asthma is mild)

Question 742

Which patients are at risk for hypoglycemia caused by a reduction in hepatic gluconeogenesis when B-blockers are used and should be monitored accordingly?

Answer 742

Infants with poor enteral intake

Question 743

What medication is contraindicated in the setting of acute decompensated heart failure?

Answer 743

B-blockers

Question 744

What makes B-blockers useful in the management of chronic heart failure?

Answer 744

Antagonism of the adverse neurohumoral modulation seen in heart failure

Question 745

Why is carvedilol a useful medication in chronic heart failure therapy?

Answer 745

Oppose adrenergic stimulation at multiple sites in addition to its B-receptor blockade

Question 746

Irregularly irregular narrow complex tachycardia?

Answer 746

Atrial fibrillation

Question 747

What is the Ashman phenomenon?

Answer 747

-Aberrant conduction secondary to a change in the tachycardia cycle length -Reason for the aberrancy is because the refractory period in cardiac cells is related to the preceding R-R interval -Usually occurs when there is a short R-R interval that follows a long R-R interval

Question 748

Atrial fibrillation most commonly occurs in pediatric patients who have what?

Answer 748

-Associated structural heart disease -WPW

Question 749

In patients without structural heart disease, some of the most common causes of atrial fibrillation include what?

Answer 749

-Thyroid abnormalities -Use of drugs of abuse

Question 750

Prevalence of atrial fibrillation in the young has been shown to be higher in which patients?

Answer 750

-Male -Obese

Question 751

Some patients that are classified as having “lone atrial fibrillation” have been found to have what as the precipitating cause of their atrial fibrillation?

Answer 751

SVT (AVNRT or concealed accessory pathways)

Question 752

What is the mechanism for SVT being the precipitating cause for atrial fibrillation?

Answer 752

-SVT degenerates into atrial fibrillation -Ablation of the underlying arrhythmia substrate has been shown to decrease risk of recurrence of atrial fibrillation

Question 753

What is the risk of recurrence in atrial fibrillation?

Answer 753

39% over 2 year follow-up period

Question 754

What features are associated with a higher risk of recurrence of atrial fibrillation?

Answer 754

-Family history of atrial fibrillation in a first degree relative -Prolonged PR interval at baseline

Question 755

Patients presenting with atrial fibrillation should be evaluated for what?

Answer 755

-Thyroid abnormalities -Drugs of abuse -Structural heart disease

Question 756

What can be the cause of atrial fibrillation in a subset of patients?

Answer 756

SVT (AVNRT or concealed accessory pathways)

Question 757

What increases the risk of recurrent atrial fibrillation?

Answer 757

-1st degree relative with atrial fibrillation -Prolonged PR interval at baseline

Question 758

A persistent PDA after birth is more common in what neonates?

Answer 758

Premature (v. those born at term)

Question 759

A persistent PDA can result in what?

Answer 759

Significant L-R shunt

Question 760

What determines the clinical significance of a PDA?

Answer 760

Volume of blood shunting across the PDA and degree of pressure restriction provided by the PDA between the aorta and PA over time

Question 761

What are the effects of a significant ductal shunt?

Answer 761

-Volume load on the pulmonary vascular bed and left heart -CHF with progressive dilation of LA/LV

Question 762

Why is increased pulmonary blood flow from a PDA a problem in a premature infant

Answer 762

-Increased pulmonary blood flow particularly deleterious to the immature pulmonary vascular bed -Results in pulmonary edema, reduced lung compliance, ineffective gas exchange -Often results in need for high ventilator settings and risk of further lung damage

Question 763

What is the recommendation for a hemodynamically significant PDA?

Answer 763

Can try medical therapy with diuretics, but definitive therapy with PDA closure is often indicated

Question 764

What are options for closure of a PDA?

Answer 764

-Pharmacologic: Indomethacin, ibuprofen, acetaminophen -Procedural: Percutaneous, surgical ligation

Question 765

What are some of the potential adverse effects of pharmacologic closure of a PDA?

Answer 765

-NEC -Kidney injury

Question 766

What is considered the standard of care for PDA that doesn’t close with pharmacologic intervention?

Answer 766

Percutaneous PDA device closure

Question 767

What is commonly seen after percutaneous closure of a PDA or surgical ligation of a PDA?

Answer 767

-Post-ligation cardiac syndrome -Dilated LA/LV with mildly depressed systolic function -Patients at risk for low CO (can occur quickly after intervention) -Develop hypotension and circulatory insufficiency

Question 768

What are risk factors for post-ligation cardiac syndrome?

Answer 768

-Large PDA -Earlier age at ligation -Lower estimated gestational age -Lower birth weight -Significant degree of pre-intervention cardiorespiratory support

Question 769

What causes post-ligation cardiac syndrome?

Answer 769

-Myocardial dysfunction and a dysregulation of vascular tone because of a sudden increase in LV afterload and decrease in LV preload -Decrease of excessive volume preload and reduction in LA filling pressure results in ventricular contractility (Frank-Starling) -Major culprit is sudden increase in afterload because LV is no longer exposed to low-resistance pulmonary vascular bed

Question 770

Why are premature infants at increased risk for post-ligation cardiac syndrome after PDA closure?

Answer 770

Premature myocardium is unable to tolerate increase in SVR from PDA closure, get resultant increase in LV systolic wall stress and decrease in LV shortening fraction and velocity of circumferential fiber shortening

Question 771

True or False: Although echo post-PDA closure can show LV systolic dysfunction, clinically relevant post-ligation cardiac syndrome is uncommon

Answer 771

True

Question 772

Why is there a reduced likelihood of post-ligation cardiac syndrome in patients who undergo percutaneous PDA closure?

Answer 772

Typically older and larger than those undergoing surgical ligation

Question 773

True or False: There is a lower incidence of post-ligation cardiac syndrome after percutaneous PDA closure in infants with extremely low birth weight compared with surgical ligation?

Answer 773

True- Percutaneous less invasive procedure likely has specific benefit itself

Question 774

What are the risks associated with transcatheter PDA device closure?

Answer 774

-Vascular damage (loss of femoral artery pulse in small infants) -Device embolization -Obstruction across the LPA or descending Ao by device

Question 775

A PDA results in what that is deleterious to the immature pulmonary vascular bed of a premature infant?

Answer 775

Increased pulmonary blood flow

Question 776

What is well described after PDA ligation surgery resulting in systemic hypotension and circulatory insufficiency?

Answer 776

Post-ligation cardiac syndrome

Question 777

What is post-ligation cardiac syndrome?

Answer 777

Involves myocardial dysfunction and a dysregulation of vascular tone because of a sudden increase in LV afterload and decrease in LV preload after ligation- particularly poorly tolerated by premature myocardium

Question 778

What has largely replaced surgical ligation as the definitive therapy for PDA closure and can be considered standard of care when nonpharmacologic intervention is required, even in very small infants?

Answer 778

Percutaneous PDA device closure

Question 779

True or False: Clinically relevant post-ligation cardiac syndrome is less common in infants with extremely low birth weights after device closure compared with surgical ligation?

Answer 779

True

Question 780

What accounts for 80-85% of cases of LVOTO?

Answer 780

Valvular aortic stenosis

Question 781

Describe the mature aortic valve

Answer 781

3 cusps/leaflets, 3 commissures

Question 782

What is the most common abnormality of the aortic valve leading to aortic stenosis?

Answer 782

Partial or complete fusion of the valve cusps

Question 783

List in order from most common to least fusion patterns of the aortic valve?

Answer 783

-Most: Left and right -Middle: Right and non -Least: Left and non

Question 784

Doppler gradients provide accurate estimates of the severity of valvular stenosis except in what situations?

Answer 784

-Decreased CO -Multiple levels of obstruction -Pop-off like a VSD *All can result in a lower Doppler gradient despite the presence of a significant stenosis

Question 785

What is considered the gold standard to measure the gradient across the aortic valve?

Answer 785

Cath

Question 786

Describe the difference between peak to peak gradients measured in the cath lab, peak instantaneous pressure gradients measured by spectral Doppler and mean Doppler gradients

Answer 786

-Measurements reflect different physiologic parameters -Peak instantaneous pressure gradient measured by spectral Doppler is higher than the peak-peak gradient measured in the cath lab -Mean Doppler gradients on echo correlate more closely with the peak-peak gradients measured in the cath lab

Question 787

What are the guidelines for treatment of AS based upon?

Answer 787

-Cath derived peak to peak gradients -Clinical symptoms -ECG changes

Question 788

A cath peak to peak gradient > what (even if asymptomatic) meets the criteria for balloon aortic valvuloplasty?

Answer 788

50

Question 789

In symptomatic patients (angina or syncope), patients with resting or exercise-induced ECG changes (ST-T changes), or in patients planning to become pregnant or participate in competitive sports, a gradient > what is recommended as the cutoff for balloon aortic valvuloplasty?

Answer 789

40

Question 790

Under what circumstance is balloon aortic valvuloplasty indicated regardless of measured pressure gradients?

Answer 790

Infants with valvular AS and depressed LV function or with critical valvar stenosis (requiring patency of the PDA for adequate systemic perfusion)

Question 791

What is often used in adults to guide decisions about aortic valvuloplasty when baseline gradients are low due to low cardiac output?

Answer 791

Drug challenge using dobutamine- but this challenge is not required in infants and children

Question 792

Balloon aortic valvuloplasty is indicated regardless of pressure gradients in what situations?

Answer 792

-Valvar AS and depressed LV systolic function -Critical valvar stenosis (requiring the patency of the ductus arteriosus for adequate systemic perfusion)

Question 793

Which is higher, peak instantaneous gradient on the echo evaluation or peak-peak gradient obtained in the cath lab?

Answer 793

Peak instantaneous gradient on echo

Question 794

What does a peak to peak gradient in the cath lab correlate more closely with on echo in valvular AS, mean gradient or peak instantaneous gradient?

Answer 794

Mean gradient

Question 795

What are skin lesions caused by abnormal deposition of lipids in the subcutaneous tissue and tendons?

Answer 795

Xanthomas

Question 796

Where are tendinous xanthomas usually located?

Answer 796

Extensor aspects of joints

Question 797

Where are subcutaneous xanthomas often found?

Answer 797

-Palmar aspects -Interdigital spaces -Around the eyes

Question 798

What are corneal arcus?

Answer 798

Lipid deposit in the cornea

Question 799

What is seen on skin biopsy of xanthomas?

Answer 799

Cholesterol crystals in the center surrounded by fibrosis and foamy macrophages

Question 800

The presence of cutaneous or tendinous xanthomas in a young child or adolescent should raise suspicion for what?

Answer 800

Inherited hyperlipidemia

Question 801

A mutation in LDL receptor gene causes what?

Answer 801

Familial hypercholesterolemia

Question 802

What is the typical lipid profile of a patient with familial hypercholesterolemia?

Answer 802

-Markedly elevated LFL -Mildly elevated TG -Normal HDL

Question 803

What are some of the genes associated with familial hypercholesterolemia?

Answer 803

-LDLR (LDL receptor) -Apo-B (apolipoprotein-b) -PCSK9 (pro-protein convertase subtilisin/kexin type 9 -LDLRAP1 (LDL receptor adapter protein 1)

Question 804

How can patients with familial hypercholesterolemia categorized?

Answer 804

-Receptor defective (2-25% residual activity) -LDL receptor deficient (<2% residual activity)

Question 805

What are the criterial for the clinical diagnosis of familial hypercholesterolemia?

Answer 805

-Genetic confirmation of 2 mutant alleles at the LDR, APOB, PCSK9 or LDLRAP1 gene loci -Untreated LDLC level >500mg/dL (13.0mmol/L) or a treated LDLC level >300mg/dL (7.8mmol/L) -In the presence of cutaneous or tendon xanthomas before the age of 10 years -Untreated elevated LDL levels in both the parents

Question 806

True or False: The usual lipid-lowering agents like statins, ezetimibe and bile acid-binding resins are ineffective in obtaining a significant reduction in the LDL-C levels

Answer 806

True

Question 807

What are 3 drugs are approved by the FDA for management of familial hypercholesterolemia (homozygous) in children >12 and adults?

Answer 807

-Lomitapide (microsomal transport protein inhibitor) -Mipomersen (antisense oligonucleotide against the apo(B) MRI -Evinacumab (monoclonal antibody that inhibits angiopoietin-like protein 3 (ANGPTL3)

Question 808

What is one medication approved by the FDA for management of familial hypercholesterolemia (homozygous) in children >10 and adults?

Answer 808

Evolocumab (PCSK9 inhibitor)

Question 809

Besides lomitapide, mipomersen, evolocumab and evinacumab what are some drugs currently being studied for use in pediatrics in familial hypercholesterolemia?

Answer 809

Inclisiran (small interfering RNA molectule (siRNA) which inhibits PCSK9 mRNA translation- FDA approved in adults, pediatric trials underway)

Question 810

What is sitosterolemia?

Answer 810

Disorder of lipid metabolism that can present with cutaneous and tendinous xanthomas in youth

Question 811

How does sitosterolemia differ from familial hyperlipidemia?

Answer 811

-AR with mutations in the ABCG5 and ABCG8 genes -Typically have normal LDL cholesterol levels -Characterized by markedly elevated levels of plant sterols in the blood with poor response to statins

Question 812

What is a first-line drug for sitosterolemia?

Answer 812

Ezetimibe (followed by bile acid binding resins and avoidance of plant sterols

Question 813

What causes neurofibromatosis?

Answer 813

Mutation in the tumor suppressor NF1 gene

Question 814

Neurofibromas + Axillary/Inguinal freckling?

Answer 814

Neurofibromatosis (mutation in tumor suppressor NF1 gene)

Question 815

What type of CHD is seen in neurofibromatosis?

Answer 815

-Pulmonary valve stenosis -Mitral valve prolapse -Septal defects -HCM

Question 816

Mutations in TSC1 or TSC2 genes cause what?

Answer 816

Tuberous sclerosis complex

Question 817

What findings are seen in tuberous sclerosis complex?

Answer 817

-Angiofibromas -Shagreen patch -Multiple café-au-lait spots -Cardiac rhabdomyomas

Question 818

What causes Fabry disease?

Answer 818

Mutation in alpha galactosidase A

Question 819

What is Fabry disease?

Answer 819

X-lined recessive lysosomal storage disorder (gene mutation causes accumulation of glycosphingolipids in the vascular endothelium, smooth muscle, heart and other organs)

Question 820

Angiokeratomas (1-2mm red papules) on trunk, perineal area and oral mucosa

Answer 820

Fabry disease

Question 821

What are the heart findings seen in Fabry disease?

Answer 821

-LVH (myocardial accumulation of the sphingolipids) -Valvular abnormalities -Systemic HTN -Atherosclerotic disease of coronary and cerebral arteries

Question 822

The presence of cutaneous or tendinous xanthomas in a young child or adolescent should raise suspicion for what?

Answer 822

Inherited hyperlipidemia

Question 823

What are the most common genetic mutations in patients with familial hypercholesterolemia?

Answer 823

-LDL receptor gene -apo-B -PCSK9 -LDLRAP1

Question 824

True or False: The usual lipid lowering agents like statins, ezetimibe, and bile acid binding resin are ineffective in obtaining a significant reduction in the LDL cholesterol levels in homozygous familial hypercholesterolemia?

Answer 824

True

Question 825

What are some of the promising new agents in lipid lowering in homozygous familial hypercholesterolemia?

Answer 825

-PCSK9 inhibitors, ANGPTL3 inhibitors, Inclisiran

Question 826

In a patient with single ventricle physiology immediately after stage I palliation with a systemic to pulmonary shunt, what governs the balance of pulmonary v. systemic blood flow (Qp:Qs)?

Answer 826

PVR and SVR

Question 827

What is the goal for a stage I single ventricle patient and how is this achieved?

Answer 827

-Balanced circulation with an equal distribution of blood flow between the pulmonary and systemic vascular beds -Manipulation in pulmonary or systemic vascular resistance

Question 828

What is needed to calculate a patient’s Qp:Qs?

Answer 828

-Systemic venous saturation -Systemic arterial saturation -Pulmonary artery saturation -Pulmonary venous saturation

Question 829

In a patient with stage I single ventricle heart disease, what assumptions can be made regarding Qp:Qs?

Answer 829

-Only source of pulmonary blood flow is shunt which receives blood flow from systemic circulation so the saturation in the PA is the same as the systemic artery -For a patient on 21% FiO2 with normal CXR, can assume that the pulmonary veins are fully saturated at close to 100%

Question 830

Equation for Qp/Qs?

Answer 830

(Systemic artery saturation – Systemic vein saturation) / (Pulmonary vein saturation – Pulmonary artery saturation)

Question 831

What are some ways you can get a sense of a patient’s cardiac output?

Answer 831

-Exam: Is patients warm/well perfused, good capillary refill, bounding pulses -AV saturation difference -Lactate level

Question 832

In a post-op cardiac patient, what can cause vasodilation leading to low SVR?

Answer 832

Rapid rewarming after CPB or peripheral vasodilators (like low-dose epinephrine and milrinone)

Question 833

How does vasopressin work?

Answer 833

Neurohypophysial hormone that directly stimulates smooth muscle V1 receptors resulting in vasoconstriction

Question 834

What is the half life of vasopressin?

Answer 834

10-20 minutes

Question 835

After starting vasopressin, you get a steady state of infusion in how long?

Answer 835

15-20 minutes

Question 836

What are the main effects of vasopressin?

Answer 836

Vasoconstriction and increased SVR

Question 837

What would be the effect of increased SVR in a stage 1 single ventricle patient?

Answer 837

Increased pulmonary blood flow through the shunt

Question 838

True or False: If you increase in SVR in a hypoxic stage 1 single ventricle, you would expect to see fairly immediate rise in O2 saturation, PaO2 and BP?

Answer 838

True

Question 839

What are the overall effects of milrinone on SVR?

Answer 839

Increase

Question 840

What is the half-life of milrinone in an infant?

Answer 840

2 hours- would take 2-4 hours before you would see an effect

Question 841

True or False: Epinephrine at 0.05ug/kg/min would increase BP if the main issue was decreased CO?

Answer 841

True

Question 842

What effects of epinephrine predominate at 0.05ug/kg/min?

Answer 842

B-agonist effects

Question 843

What effects of epinephrine would help to increase SVR?

Answer 843

Alpha-agonist

Question 844

What are the effects of lower dose v. higher dose epinephrine on SVR?

Answer 844

-Lower dose (0.05ug/kg/min), B-agonist effects predominant, not likely to increase SVR -Higher doses, alpha-agonist effects predominate, increase SVR

Question 845

Effect of iNO on PVR?

Answer 845

Decrease

Question 846

In addition to lowering PVR, what other effect does sildenafil have?

Answer 846

Decreases SVR- can cause hypotension (need to be cautious with use in single ventricles especially if trying to augment Qp:Qs)

Question 847

In a stage 1 single ventricle with low SpO2 and normal CO, what could be the underlying pathophysiology?

Answer 847

Low SVR

Question 848

In a stage 1 single ventricle with increased SpO2 and poor perfusion/CO, what could be the underlying pathophysiology?

Answer 848

Decreased PVR

Question 849

How can prolonged low CO after CPB lead to AKI?

Answer 849

Suboptimal hemodynamics lead to a low renal perfusion pressure, decreased GFR and decreased urine output

Question 850

What are the goals in the ICU for a patient in low CO state after CPB?

Answer 850

Preserve end-organ function and perfusion to prevent injury and promote recovery as the myocardial function recovers

Question 851

What is stage 2 AKI by pediatric kidney disease improving global outcomes criteria?

Answer 851

<0.5mL/kg/hr of UOP over 12 hours

Question 852

What are some of the categories to evaluate to diagnose AKI?

Answer 852

-Urine output -Rise in creatinine above baseline -Emerging biomarkers (Neutrophil gelatinase associated lipocalin (NGAL) or cystatin-C)

Question 853

What should be done to promote a higher renal perfusion pressure in a patient with high venous pressures and low urine output?

Answer 853

Reduce CVP- fluid removal

Question 854

What can help to augment fluid removal when a patient is diuretic resistant?

Answer 854

Advanced renal replacement therapies

Question 855

Why is post-op fluid removal associated with better outcomes?

Answer 855

-Improve end organ perfusion pressure -Improve cardiac function by improving the Starling curve mechanics

Question 856

What are risk factors for cardiac surgery-associated AKI?

Answer 856

-Neonatal age -Lower weight -Exposure to CPB -Exposure to nephrotoxic agents -Low CO syndrome -Higher preoperative illness severity

Question 857

What is often the first-line renal replacement therapy when other medical therapies have failed in neonates with cardiac surgery associated AKI?

Answer 857

Peritoneal dialysis (some center empirically place a PD drain in certain neonates in OR)

Question 858

What are 2 ways a PD drain can be used?

Answer 858

-Passive drain for fluid removal -Dialysate fluid instilled and dwelled prior to draining to perform dialysis

Question 859

True or False: In many cases, a patient will only require a few says of peritoneal dialysis before AKI resolves in cardiac surgery associated AKI?

Answer 859

True

Question 860

What can be used to augment vasoactive therapies in the post-op patient (particularly in catecholamine-resistant vasoplegic shock)?

Answer 860

Corticosteroids

Question 861

True or False: Fluid overload should be avoided and corrected in the context of a patient’s underlying disease to avoid complications and need for increased resource utilization

Answer 861

True

Question 862

In neonates with diuretic refractory fluid overload in the setting of AKI, what is often a first-line therapy for fluid removal?

Answer 862

Peritoneal dialysis

Question 863

True or False: A RAA with an aberrant left subclavian artery and a left-sided ductus is an anatomic substrate for vascular ring

Answer 863

True

Question 864

What is a vascular ring?

Answer 864

Anomaly where the trachea and esophagus are encircled by vascular structures

Question 865

What are the effects of a vascular ring?

Answer 865

External compression with variable respiratory or GI symptoms depending on the degree of compression

Question 866

What is the clinical evaluation for a vascular ring driven by?

Answer 866

Patients symptom burden- Majority of postnatally discovered rings are diagnosed in symptomatic patients

Question 867

In surgical series, what type of vascular ring comprises the largest proportion of cases and why?

Answer 867

Double aortic arch- ring is tighter and compression is more severe (as compared to right aortic arch with an anomalous left subclavian)

Question 868

What is the most common type of vascular ring seen in prenatal studies?

Answer 868

Right aortic arch with anomalous left subclavian artery (80% v. 20% double aortic arch)

Question 869

True or False: Patients with right aortic arch and anomalous left subclavian artery are less likely to be symptomatic than double aortic arch?

Answer 869

True- surgical series show more double aortic arch, but prenatal series show more RAA-ALSCA (more people with RAA-ALSCA, but less likely to cause problems and need surgery, lack of significant symptoms postnatally) *Recent study showed 33% of RAA-ALSCA patients diagnosed in utero had symptoms versus 75% of patients diagnosed in utero with DAA

Question 870

True or False: Symptom onset is earlier and more severe in double aortic arch than right aortic arch with anomalous left subclavian artery

Answer 870

True

Question 871

Which type of symptoms, respiratory (stridor, wheezing) or GI (reflux, vomiting, swallowing-related), are more common in double aortic arch?

Answer 871

Respiratory (ratio of 2-4:1)

Question 872

Surgical repair is indicated for which patients with vascular ring?

Answer 872

Symptomatic (asymptomatic patients can be followed expectantly)

Question 873

What 2 things does the natural history of vascular ring vary by?

Answer 873

-Anatomic substrate -Timing of diagnosis (prenatal or postnatal

Question 874

True or False: A patient with a fetal diagnosis of isolated right aortic arch with an aberrant left subclavian artery is likely to be asymptomatic

Answer 874

True