B P9 C82 CHD in the Adolescent and Adult

Question 1

At least __% of these adult congenital heart disease (ACHD) patients have complex cardiovascular anatomy and suffer from multi-system organ involvement.

Answer 1

20%

Question 2

Transition discussions should begin at the age of __ years, with the expectation of complete transfer to ACHD care by __ years of age.

Answer 2

Begin at 12 years
Complete transfer at 21 years

Question 3

Anatomic Classification of CHD (Class I)

Answer 3

Class I: Simple

Native disease
* Isolated small ASD
* Isolated small VSD
* Mild isolated pulmonic stenosis

Repaired conditions
* Previously ligated or occluded ductus arteriosus
* Repaired secundum ASD or sinus venosus defect without significant residual shunt or chamber enlargement
* Repaired VSD without significant residual shunt or chamber enlargement

Question 4

Anatomic Classification of CHD (Class II)

Answer 4

Class II: Moderate Complexity

• Aorto-left ventricular fistula
• Anomalous pulmonary venous connection, partial or total
• Anomalous coronary artery arising from the pulmonary artery
• Anomalous aortic origin of a coronary artery from the opposite sinus
• AVSD (partial or complete, including primum ASD)
• Congenital aortic valve disease
• Congenital mitral valve disease
• Coarctation of the aorta
• Ebstein anomaly (disease spectrum includes mild, moderate, and
  severe variations)
• Infundibular right ventricular outflow obstruction
• Ostium primum ASD
• Moderate and large unrepaired secundum ASD
• Moderate and large persistently patent ductus arteriosus
• Pulmonary valve regurgitation (moderate or greater)
• Pulmonary valve stenosis (moderate or greater)
• Peripheral pulmonary stenosis
• Sinus of Valsalva fistula/aneurysm
• Sinus venosus defect
• Subvalvar aortic stenosis (excluding HCM; HCM not addressed in
  these guidelines)
• Supravalvar aortic stenosis
• Straddling atrioventricular valve
• Repaired tetralogy of Fallot
• VSD with associated abnormality and/or moderate or greater shunt

Question 5

Anatomic Classification of CHD (Class III)

Answer 5

Class III: Great Complexity (or Complex)

• Cyanotic congenital heart defect (unrepaired or palliated, all forms)
• Double-outlet ventricle
• Fontan procedure
• Interrupted aortic arch
• Mitral atresia
• Single ventricle (including double inlet left ventricle, tricuspid atresia, hypoplastic left heart, any other anatomic abnormality with a functionally single ventricle)
• Pulmonary atresia (all forms)
• TGA (classic or d-TGA; CCTGA or
  l-TGA)
• Truncus arteriosus
• Other abnormalities
  of atrioventricular and ventriculoarterial connection
  (i.e., crisscross heart, isomerism, heterotaxy syndromes, ventricular inversion)

Question 6

Physiologic stages of ACHD patient classification

Answer 6

A: NYHA FC I, normal exercise capacity, normal renal, hepatic, and pulmonary function
B: NYHA FC II, mild valvular disease, arrhythmias not requiring treatment, mild hemodynamic sequelae, abnormal objective cardiac limitation to exercise, trivial or small shunt
C: NYHA FC III, significant valvular disease, arrhythmias controlled with treatment, moderate or greater ventricular dysfunction, moderate aortic enlargement, venous or arterial stenosis, mild or moderate hypoxemia/cyanosis, hemodynamically significant shunt, pulmonary hypertension, end-organ dysfunction responsive to therapy
D: NYHA FC IV, arrhythmias refractory to treatment, severe aortic enlargement, severe hypoxemia, severe pulmonary hypertension, Eisenmenger syndrome, refractory end-organ dysfunction

Question 7

Location of surgical scars will indicate whether a patient has had a lateral thoracotomy, such as with a _____.

Answer 7

Patent ductus arteriosus (PDA) ligation

or

Aortic coarctation repair

Question 7

Absence of a radial pulse on the ipsilateral arm as a thoracotomy scar may suggest that the subclavian artery was sacrificed in the repair, such as a _____.

Answer 7

Subclavian flap repair for coarctation of the aorta

or

Prior classic Blalock-Taussig-Thomas [BTT] shunt

Question 8

Anomalous left coronary artery from the pulmonary artery (ALCAPA)

Answer 8

Bland-White-Garland syndrome

Question 9

Pulmonary hypertension with cyanosis due to right to left shunting

Answer 9

Eisenmenger Syndromer

Question 10

Septal defect resulting in direct left ventricle to right atrium shunt

Answer 10

Gerbode Syndrome

Question 11

Double inlet left ventricle with D-looped ventricles and normally related great vessels

Answer 11

Holmes Heart

Question 12

Coronary sinus septal defect in the presence of a left superior vena cava

Answer 12

Raghib Defect

Question 13

Partial anomalous pulmonary venous connections of the right lower pulmonary vein to the IVC-RA junction, often accompanied by pulmonary artery hypoplasia and aortopulmonary collateral formation.

Answer 13

Scimitar Syndrome

Question 14

Syndrome with a series of left-sided obstructive lesions

Answer 14

Shone Syndrome

Question 15

Form of double outlet right ventricle with D-malposed, side-by-side great vessels, sub-pulmonary VSD, hypoplastic aortic arch

Answer 15

Taussig-Bing Malformation

Question 16

Early palliative procedure for transposition of the great arteries, with the inferior vena cava directed to the left atrium via homograft

Answer 16

Baffes Procedure

Question 17

“Classic”—direct end to end anastomosis of subclavian artery to pulmonary artery

“Modified”—tube graft from subclavian artery to pulmonary artery

Answer 17

Blalock-Taussig (-Thomas) Shunt

Question 18

Closed infundibular resection for relief of pulmonary stenosis

Answer 18

Brock Procedure

Question 19

Atriopulmonary anastomosis for single ventricle heart disease

Answer 19

Fontan or Fontan-Kreutzer

Question 20

Includes the right ventricle into the pulmonary circulation, was the unique modification for tricuspid atresia

Answer 20

Fontan-Björk Modification

Question 21

“Classic”—end to end anastomosis of superior vena cava to right pulmonary artery

“bidirectional”—end to side anastomosis of superior vena cava to right pulmonary artery

Answer 21

Glenn

Question 22

Bidirectional Glenn in context of interrupted inferior vena cava with azygos continuation to the superior vena cava

Answer 22

Kawashima

Question 23

Anterior translocation of the pulmonary arteries, so that both branch pulmonary arteries run anterior to the aorta. Most commonly used as part of the arterial switch operation

Answer 23

Lecompte Maneuver

Question 24

Atrial switch operations for transposition of the great arteries, with atrial baffling using native atrial or pericardial tissue to redirect systemic and pulmonary venous flow

Answer 24

Atrial: Senning Pericardial: Mustard

Question 25

In double outlet right ventricle, posterior translocation of the aortic root towards the left ventricle, with baffling of the left ventricle to the aorta in its new position

Answer 25

Nikaidoh

Question 26

Neonatal palliative procedure for hypoplastic left heart syndrome including aortic arch reconstruction with anastomosis of the native aorta to the pulmonary artery, which becomes the “neo-aorta,” as well as atrial septectomy and a modified BT shunt

Answer 26

Norwood

Question 27

Direct anastomosis of the left pulmonary artery to the descending aorta

Answer 27

Potts Shunt

Question 28

Intra-cardiac routing of the left ventricle to the aorta, which arose from the right ventricle. Usually accompanied by a right ventricle to pulmonary artery conduit.

Answer 28

Rastelli

Question 29

Intrapulmonary baffle of the left coronary artery performed for anomalous left coronary artery from the pulmonary artery

Answer 29

Takeuchi Repair

Question 30

Direct anastomosis of the right pulmonary artery to the ascending aorta

Answer 30

Waterson shunt

Question 31

Fixed S2 splitting

Answer 31

ASD

Question 32

Diminished lower extremity pulse

Answer 32

Aortic coarctation

Question 33

A classic physical examination finding in a patient with _____ is a systolic ejection click which decreases in intensity with inspiration.

Answer 33

Pulmonary stenosis

Question 34

This remains the cornerstone of cardiac imaging in the CHD patient

Answer 34

Echocardiography

Question 35

This is recommended in any ACHD patient with signs of elevated pulmonary artery (PA) pressure to determine pulmonary vascular resistance (PVR)

Answer 35

Cardiac Catheterization

Question 36

Abnormal lung function is com- mon in ACHD patients,and up to __% of ACHD patients have abnormal pulmonary function tests.

Answer 36

40%

Question 37

Multiple mechanisms for abnormal pulmonary mechanics in ACHD patients,

Answer 37

RLD: post-op patients Diaphragmatic paralysis: phrenic nerve injury Asymmetric pulmonary blood flow due to branch PA abnormalities or acquired conditions

Question 38

Hemoptysis may occur in up to _____of ACHD patients with Eisenmenger syndrome.

Answer 38

1/3

Question 39

PH is found in up to __% of ACHD patients and is strongly associated with increased morbidity and mortality

Answer 39

10%

Question 40

This is common in ACHD patients and has been shown to be a primary driver of high-resource utilization for ACHD hospitalizations,accounting for up to one-third of hospital charges

Answer 40

Renal dysfunction

Question 41

_____-based estimated glomerular filtration rate (eGFR) more accurately predicts clinical effects in ACHD patients than creatinine-based eGFR.

Answer 41

Cystatin C

Question 42

The majority of the evidence of hepatic dysfunction in ACHD patients has been focused on _____

Answer 42

Fontan-associated liver disease (FALD)

Question 43

_____ remains an important cause of liver disease in older ACHD patients, particularly those who received blood transfusions prior to 1992.

Answer 43

Hepatitis C

Question 44

Abnormalities of the coagulation system are observed in patients with _____ and are associated with bleeding and thrombotic complications.

Answer 44

Fontan physiology

Question 45

Cancer is the second leading cause of non- cardiovascular death in ACHD patients, with certain malignancies having an increased prevalence in specific CHD conditions, that is, _____.

Answer 45

HCC in adults with Fontan physiology

Question 46

It is the most common aneuploidy and is usually caused by trisomy 21.

Answer 46

Down Syndrome

Question 47

It is also the most common chromosome abnormality associated with CHD

Answer 47

Down Syndrome

Question 48

Fifty percent of children born with Down syndrome have CHD, most commonly defects in the _____

Answer 48

AV canal

Question 49

Sinus node dysfunction is most commonly related to _____.

Answer 49

Surgical repair

Question 50

Sinus node dysfunction is commonly encountered in patients with _____.

Answer 50

D-loop TGA who have undergone a Mustard or Senning operation Repair for ASD or sinus venosus defects

Question 51

_____ pacing is required for symptomatic sinus node dysfunction.

Answer 51

Atrial pacing

Question 52

Patients who have had a _____ and those with open atrial shunts typically require epicardial pacing.

Answer 52

Fontan operation with an extracardiac conduit

Question 53

Heart block is common in patients with _____ as the AV node is superiorly and anteriorly displaced

Answer 53

L-loop TGA

Question 54

_____ is the most common tachyarrhythmia in CHD, accounting for 62% of atrial arrhythmias.

Answer 54

Interatrial re-entrant tachycardia (IART)

Question 55

In patients who have undergone the atrial switch operation, IART is a dangerous arrhythmia which may convert to _____ as the noncompliant interatrial baffles and systemic RV perform poorly at high heart rates

Answer 55

Polymorphic VT or PEA

Question 56

_____ control is usually preferred for adults with CHD and IART.

Answer 56

Rhythm

Question 57

_____ are often preferred in patients with complex CHD. Amiodarone has considerable cumulative toxicity so is usually not a preferred first-line anti- arrhythmic in younger patients but is sometimes required for patients with ventricular dysfunction.

Answer 57

Sotalol and dofetilide

Question 58

Patients with _____ and IART should receive long-term anticoagulation.

Answer 58

Fontan circulation

Question 59

As patients with CHD age, ____ becomes more common and is the most common atrial arrhythmia in adults with CHD older than age 50.

Answer 59

Atril Fibrillation

Question 60

_____ is the second most common cause of cardiac death in adults with CHD following heart failure,and accounts for approximately 20% of death in patients with CHD, occurring at a rate of ∼0.1% per patient year.

Answer 60

Sudden Death

Question 61

_____, and complex forms of CHD are at the highest risk for ventricular arrhythmias and sudden cardiac death.

Answer 61

TOF TGA with a systemic RV Fontan circulation Eisenmenger syndrome

Question 62

Pacemakers should be _____ in patients with intracardiac shunts, single ventricle, or ventricular leads in Fontan circulation.

Answer 62

Epicardial

Question 63

Class I recommendations for secondary prevention with ICDs in adults with CHD

Answer 63

Class IB: ICD therapy is indicated in adults with CHD who are survivors of cardiac arrest due to ventricular fibrillation or hemodynamically unstable VT after evaluation to define the cause of the event and exclude any completely reversible etiology Class IB: ICD therapy is indicated in adults with CHD and spontaneous sustained VT who have undergone hemodynamic and electrophysiologic evaluation. Class IC: Catheter ablation or surgery may offer a reasonable alternative or adjunct to ICD therapy in carefully selected patients

Question 64

Class I recommendations for primary prevention with ICDs in adults with CHD

Answer 64

Class IB: ICD therapy is indicated in adults with CHD and a systemic left ventricular ejection fraction ≤ 35%, biventricular physiology, and NYHA Class II or III symptoms

Question 65

Heart failure is most common in patients with high anatomic or physiologic complexity including:

Answer 65

Single ventricle anatomy Systemic RV PH Cyanosis

Question 66

The systemic RV is predisposed to systolic dysfunction due to:

Answer 66

Unfavorable myocardial fiber orientation Nonconical shape Coronary supply-demand mismatch Volume loading from tricuspid regurgitation

Question 67

Fontan failure is characterized by:

Answer 67

Chronically elevated central venous pressure Low cardiac output Cyanosis due to collaterals Ascites Cirrhosis

Question 68

Standard guideline-directed medical therapy (GDMT) is likely effective when taken by CHD patients with a _____.

Answer 68

Systemic LV who have heart failure due to left ventricular systolic dysfunction after residual hemodynamic lesions have been addressed

Question 69

CRT via multi-site pacing is appropriate for patients with a _____.

Answer 69

Systemic LV Spontaneous or pacing-induced left bundle-branch block Heart failure

Question 70

Placing CRT leads is technically more difficult in patients with a systemic RV due to _____. It is reasonable to consider placement of epicardial multi-site pacing leads in selected patients if they are going for cardiac surgery for other indications.

Answer 70

Variability in the location of the CS os Variant coronary vein anatomy Presence of surgical interatrial baffles

Question 71

Patients with CHD and heart failure refractory to medical therapy should be considered for ____.

Answer 71

Heart Transplantation

Question 72

Compared to patients with acquired heart failure, CHD patients listed for heart transplantation are _____.

Answer 72

Younger More likely to have a prior sternotomy Less likely to have coronary artery disease Less likely to have a left ventricular assist device

Question 73

The current multimodality imaging guidelines for the thoracic aorta in adults advocate obtaining aortic root measurements at _____ with a leading edge–to–leading edge technique, whereas imaging guidelines for pediatric echocardiograms, which are often used in echocardiography labs specializing in CHD, suggest obtaining aortic root measurements in _____ with an inner edge–to–inner edge technique.

Answer 73

End-diastole Mid-systole

Question 74

Aortic dilation is common in adults with CHD, particularly in patients with _____.

Answer 74

BAV Conotruncal defects

Question 75

Antibiotic prophylaxis is recommended prior to dental procedures for ACHD patients with high-risk characteristics, which include:

Answer 75

(1) prior episodes of SBE (2) prosthetic valves (including transcatheter) (3) valve repair using a prosthetic ring (4) residual intracardiac shunts adjacent to prosthetic material (5) cyanotic CHD (6) any CHD repaired with prosthetic material up to 6 months after the procedure or lifelong if residual shunt or valvular regurgitation remains (7) for cardiac transplant recipients who develop cardiac valvulopathy

Question 76

The most common pathogens responsible for SBE include:

Answer 76

Streptococcus viridans Staphylococcus species Enterococcus species

Question 77

The CARPREG 2 investigators reported the mater- nal outcomes of 1938 pregnancies in women with cardiac disease (63% CHD), and 16% of women experienced an adverse cardiac out- come, primarily heart failure and arrhythmias. The highest-weighted risk factors (weight of three points) include:.

Answer 77

(1) A prior history of cardiac events or arrhythmias (2) Decreased functional status (New York Heart Association, NYHA, Class ≥III) (3) Presence of a mechanical heart valve

Question 78

CARPREG2 The predicted risks for cardiac events stratified according to point score were:

Answer 78

≤1 point (5%) 2 points (10%) 3 points (15%) 4 points (22%), >4 points (41%)

Question 79

The recommended follow-up for women with WHO risk category II is ____; women with WHO risk category ≥III should be seen _____.

Answer 79

WHO risk category II: every trimester WHO risk category ≥III: monthly or bimonthly

Question 80

Women with a high risk of maternal morbidity or mortality, including women with _____ should be counselled against pregnancy and consider termination if they become pregnant

Answer 80

Pulmonary arterial hypertension (PAH) Eisenmenger syndrome Severe systemic ventricuar dysfunction Severe left-sided obstructive lesions Physiological stage D

Question 81

Low-risk patients can begin a training program at approximately __% maximal predicted heart rate at least three times per week (totaling 150 minutes/week) and increase intensity or duration over time.

Answer 81

70% Congenital conditions in which high intensity exercise should be avoided: Cyanosis High risk coronary anomalies Hypoxemia Severe aortic dilation Severe outflow tract obstruction Severe pulmonary hypertension Severe ventricular dysfunction Ventricular arrhythmias

Question 82

The degree of left-to-right shunting determines the amount of chamber dilation and is dictated by the _____.

Answer 82

(1) Size of the defect (2) Diastolic properties of the heart (3) Resistance in the great arteries

Question 83

In general, shunt lesions proximal to the tricuspid valve (such as ASDs and anomalous pulmonary venous return) cause _____ dilation, those below the tricuspid valve (such as VSDs and PDAs) cause left heart dilation

Answer 83

Proximal to TV: Right heart dilation Below the TV: Left heart dilation

Question 84

The most common type of ASD is a _____ ASD, which is a true deficiency in the atrial septum, in the region of the fossa ovalis.

Answer 84

Secundum ASD

Question 85

Secundum ASDs should be differentiated from a _____, which is persistence of patency of the flap valve of the fossa ovalis (not associated with right-sided cardiac dilation) and persists in up to 25% of adults

Answer 85

Patent foramen ovale (PFO)

Question 86

_____ ASDs may be considered in the spectrum of AVSDs and involve a deficiency in the region of the AV valves and are associated with a cleft in the mitral valve

Answer 86

Primum ASD

Question 87

Sinus venosus defects occur in the sinus venosus septum posterior to the true atrial septum and usually involve _____.

Answer 87

Anomalous right-sided pulmonary venous return

Question 88

CS defects (also called unroofed CSs) are rare and involve direct communication of the CS and the LA due to complete or partial unroofing of the CS and are often accompanied by a _____.

Answer 88

Persistent left-sided SVC

Question 89

PAPVC may be associated with either right- or left-sided pulmonary veins which can have several possible anomalous connections, with the most common being a _____.

Answer 89

LUPV to an ascending vertical vein into the brachiocephalic vein or RUPV draining to the SVC

Question 90

When the right-sided pulmonary veins connect to the inferior vena cava (IVC), this is called a _____.

Answer 90

Scimitar Vein

Question 91

The presence of cyanosis in patients with ASD should alert one to the possibility of _____.

Answer 91

(1) Shunt reversal and Eisenmenger syndrome OR (2) Prominent eustachian valve directing the inferior vena caval flow to the LA via a secundum ASD or sinus venosus defect of the inferior vena caval type

Question 92

The classic physical examination of an ASD is a _____, which is due to prolonged RV ejection and increased PA capacitance, which, in turn, delay pulmonary valve closure.

Answer 92

Wide, fixed splitting of the S2

Question 93

The ECG in ASD commonly displays a _____.

Answer 93

Rightward QRS axis and an incomplete RBBB

Question 94

The classic chest radiograph features are of _____.

Answer 94

Cardiomegaly (from right atrial and right ventricular enlargement) Dilated central pulmonary arteries with pulmonary congestion

Question 95

When an ASD is discovered in an adult, if there is any degree of right heart dilation associated with symptoms, closure should be consid- ered.51 It is important to verify the direction of the shunt as left-to-right. The next step is to verify that the _____.

Answer 95

(1) PVR is less than 1⁄3 the SVR (2) PA systolic pressure is less than 50% systemic (3) Right heart enlargement (4) Qp/Qs is at least 1.5:1

Question 96

Special attention must be paid to those defects with partial anomalous pulmonary venous drainage, as redirecting the pulmonary venous flow may result in _____

Answer 96

Pulmonary vein stenosis

Question 97

In patients with primum ASDs, care must be taken in closing the mitral valve cleft to avoid ____.

Answer 97

Mitral stenosis or residual regurgitation

Question 98

The imaging recommendations for patients following transcatheter ASD closure are dictated by the specific manufacturer, but in general, TTE is performed at _____.

Answer 98

1 week, 1 month, and then annually for at least 5 years following closure

Question 99

Any patient who has had a transcatheter ASD device placed who presents with chest pain should have an urgent evaluation to rule out _____, which occurs in 1 in 1000 cases.

Answer 99

Device erosion

Question 100

AVSDs are the result of failed fusion of the _____, and comprise up to 5% of CHD

Answer 100

Endocardial cushions

Question 101

Complete AVSDs are characterized by both an atrial and ventricular level defect with a common AV valve, usually comprised of ____ leaflets

Answer 101

Five

Question 102

A partial AVSD does not have a VSD component, and almost always has a _____.

Answer 102

Cleft in the anterior mitral valve leaflet

Question 103

A unique anatomic feature in patients with AVSD is _____, due to the **aortic valve being displaced anteriorly**.This results in a **scooped-out appearance of the ventricular septum and a shortened LV inlet**, creating a “goose neck” appearance.

Answer 103

Elongation of the LVOT

Question 104

Indications for surgical intervention of AVSD in adults are similar to those for ASD closure. These include _____.

Answer 104

(1) a net left-to-right shunt (Qp:Qs ≥1.5:1) (2) PA systolic pressure less than 50% systemic (3) PVR less than one third systemic

Question 105

For adults with prior AVSD repair, left ____ is the most common reason for later surgical reintervention.

Answer 105

AV valve regurgitation

Question 106

_____ are the most common form of CHD, and the reported incidence of isolated varies widely, from 1.5 to 53 per 1000 live births.

Answer 106

VSDs

Question 107

Many _____ VSDs are small and close spontaneously in childhood.

Answer 107

Isolated muscular and perimembranous VSDs

Question 108

Continuous wave Doppler through these defects reveals a high velocity, often greater than ____ msec, which confirms the pressure-restrictive physiology. In this case, the left-to-right shunt is minimal, and there is usually not left ventricular dilation

Answer 108

5 msec

Question 109

Angiogram of the left ventricular outflow tract demonstrating the classic “gooseneck” deformity in a patient with a _____. This appearance is the result of the inlet dimension of the left ventricle being shorter than the outlet dimension

Answer 109

Primum ASD

Question 110

Adults with a VSD and evidence of _____ should undergo VSD closure if PA systolic pressure is less than 50% systemic and PVR is less than one third systemic (AHA/ACC Class I recommendation, level of evidence B-NR).

Answer 110

LV volume overload and hemodynamically significant shunts (Qp:Qs ≥ 1.5:1)

Question 111

The gold standard for VSD closure remains _____. However, transcatheter device occlusion of selected _____ defects is possible with favorable outcomes.

Answer 111

Surgical direct suture closure or patch Muscular and perimembranous

Question 112

The ductus arteriosus, derived from the _____, connects the PA to the descending aorta in fetal life and normally closes within the first day of life.

Answer 112

Distal left sixth primitive arch

Question 113

Measurement of oxygen saturation should be performed in feet and both hands in adults with a PDA to assess for the presence of right-to-left shunting, as _____ is caused by right-to-left shunting in PDA predominantly downstream from the ductal insertion into the aorta

Answer 113

Cyanosis

Question 114

On auscultation, a classic murmur of PDA is heard best just below the ____ and typically extends from _____ into diastole and peripheral pulses may be bounding.

Answer 114

Below the left clavicle Extends from systole past the second heart sound into diastole

Question 115

PDA closure in adults is recommended if:

Answer 115

Left atrial or LV enlargement is present and attributable to PDA Net left-to-right shunt PA systolic pressure is less than 50% systemic, and PVR less than one third systemic

Question 116

Patients with device occlusion or after surgical closure should be examined periodically for possible _____

Answer 116

Recanalization

Question 117

Endocarditis prophylaxis is recommended for _____ months following PDA device closure for life if any residual defect persists following device closure

Answer 117

6 months

Question 118

Ebstein anomaly is characterized by apical displacement of the _____ from the AV junction into the RV resulting from failure of delamination of the tricuspid valve leaflets from the underlying RV endocardium during cardiac development

Answer 118

Septal tricuspid valve leaflets

Question 119

A distance between insertion sites of the two AV valves greater than _____ mm/m2 is required for the diagnosis of Ebstein malformation of the tricuspid valve, or a maximum displacement of more than ___ mm in an adult.

Answer 119

8mm/m2 Max displacement in an adult: 20 mm

Question 120

Patients with Ebstein anomaly are typically followed lifelong on a _____ depending on their clinical status

Answer 120

Yearly basis or less frequently

Question 121

Those with Ebstein anomaly and _____ require closer follow-up.

Answer 121

Impaired RV function Cyanosis Cardiomegaly Recurrent atrial arrhythmia, Severe tricuspid regurgitation after tricuspid valve repair Dysfunctional tricuspid valve bioprosthetic or mechanical valves

Question 122

Patients with Ebstein anomaly with a normal jugular venous pressure is usual at physical examination even with significant tricuspid regurgitation because of the _____.

Answer 122

Large and compliant RA and atrialized RV

Question 123

ECG findings in Ebstein anomaly:

Answer 123

(1) Low voltage QRS (2) Peaked tall P waves (>2.5 mm) in leads II and VI due to right atrial enlargement or “Himalayan” p waves (>5 mm height) (3) Prolonged PR interval (4) Importantly a short PR interval and delta wave reflective of pre-excitation from an accessory pathway

Question 124

The chest radiograph in Ebstein anomaly may show cardiomegaly, decreased pulmonary vascular markings, a small aorta and pulmonary trunk shadow, and may classically be described as showing a "____"heart.

Answer 124

Box shaped heart

Question 125

Use of CMR in Ebstein anomaly

Answer 125

(1) degree of displacement and rotation of the tricuspid valve (2) quantify tricuspid regurgitation, RV volumes and RV systolic function,LV volumes which may be small, cardiac output which may be low (3) Qp:Qs for measurement of cardiac shunt

Question 126

Natural history outcomes for 72 adults with unoperated Ebstein anomaly demonstrated decreased survival due to biventricular failure or sudden death which were predicted by:

Answer 126

Younger age at diagnosis Male gender Increased cardiothoracic ratio ≥0.65 Severity of valve displacement

Question 127

Atrial arrhythmia in Ebstein anomaly increases in prevalence with age. _____is the most common.

Answer 127

Atrioventricular reentrant tachycardia (AVRT)

Question 128

It is preferable to treat arrhythmia with _____ before surgery as following tricuspid valve surgery access to right-sided accessory pathways and the slow pathway in AV node re-entry tachycardia may be hindered

Answer 128

Ablation

Question 129

The goal of surgery in Ebstein anomaly is to:

Answer 129

(1) increase pulmonary blood flow (2) minimize tricuspid regurgitation (3) eliminate interatrial shunting (4) improve RV function

Question 130

Surgery for Ebstein anomaly is recommended for:

Answer 130

(1) symptoms (2) heart failure attributed to tricuspid valve disease (3) exercise intolerance demonstrated by exercise testing (4) progressive systolic dysfunction

Question 131

____ is favorable for Ebstein anomaly repair.

Answer 131

(1) A large mobile anterior leaflet with a free leading edge and (2) sufficient septal leaflet tissue

Question 132

Tricuspid valve _____________ is preferred when feasible.

Answer 132

Repair

Question 133

Pulmonic valve stenosis severity Mild Moderate Severe

Answer 133

Mild stenosis less than or equal to 36 mm Hg (3 msec) Moderate - 36 to 64 mm Hg (3 to 4 msec) Severe - greater than 64 mm Hg (4 msec)

Question 134

Cardiovascular exam of patients with isolated PS will include a crescendo-decrescendo systolic ejection murmur of variable intensity and often a systolic ejection click. This click becomes softer with _______________.

Answer 134

Inspiration

Question 135

Give indications for intervention in PS: This is the preferred intervention for isolated PS

Answer 135

Class I indication for moderate or severe valvar p nary stenosis in the presence of symptoms including heart failure, exercise intolerance, or cyanosis. For asymptomatic patients with severe valvar pulmonary stenosis, intervention is considered reasonable, as a class IIa indication. Primary intervention by **balloon valvuloplasty** is recommended, with surgical repair recommended for patients who have a contraindication to or have failed the transcatheter intervention.

Question 136

______________ is the most common cyanotic congenital heart defect, comprising 1. 2. 3. 4

Answer 136

TOF is the most common cyanotic congenital heart defect, comprising a “tetrad” of overriding aorta, VSD, pulmonary stenosis, and right ventricular hypertrophy.

Question 137

Associated anomalies include right aortic arch, present in about 25% of patients; anomalous course of the coronary arteries, the most common with a __________________________ and crosses the RVOT.

Answer 137

Left anterior descending artery that originates from the right coronary artery

Question 138

_______________________ is a common sequela in repaired TOF patients particularly in patients who received a transannular patch repair

Answer 138

Pulmonary regurgitation

Question 139

Pulmonary valve replacement in TOF is considered when RV end-systolic volume indexed to body surface area reaches ________ (or end-diastolic volume index reaches ______________)

Answer 139

ESVi 80 ml/m2 EDVi 160 ml/m2 These RV volume thresholds predict normalization of RV volume following intervention

Question 140

___________________ is the most common cause of death in patients with repaired TOF.

Answer 140

Heart failure is the most common cause of death in patients with repaired TOF

Question 141

In D-loop TGA, there is ventriculo-arterial discordance, which means

Answer 141

The aorta arises from the RV and the PA arises from the LV The vast majority of patients with TGA, the atria are normally positioned and the AV connections are normal. Venous return is typically normal. The great arteries are transposed with the aortic valve anterior and rightward to the pulmonic valve (D-loop transposition). In some, the aortic valve is directly anterior to the pulmonic valve or the valves can be side-by-side.

Question 142

35% of patients with TGA have this associated congenital anomaly

Answer 142

VSD

Question 143

Type of surgical repair The SVC and IVC are re-routed leftward toward the mitral valve and the LV via inter-atrial baffles. The pulmonary veins are re-routed anteriorly and rightward, toward the tricuspid valve and the RV.

Answer 143

Atrial Switch Operation RV is the systemic ventricle, the tricuspid valve is the systemic AV valve, and the LV and mitral valve support the pulmonary circulation

Question 144

Type of surgical repair This transects the great arteries above the sinuses and anastomoses them to the contralateral root. The coronary arteries must be removed from the native aortic root and reanastomosed to the neo-aortic root,

Answer 144

Arterual Switch Operation In the contemporary era the arterial switch operation is the preferred treatment for TGA Following the arterial switch o ation, the LV supports the systemic circulation and the RV supports the pulmonary circulation

Question 145

Type of surgical repair This over-sews the native pulmonic valve and places an angled VSD patch to direct oxygenated blood from the LV, across the VSD, and to the aortic valve. A RV-to-PA conduit provides for pulmonary blood flow

Answer 145

Rastelli Operation

Question 146

Baffle malfunction such as narrowing commonly involves the __________ ____________ communication between the systemic venous pathway and the pulmonary venous pathway

Answer 146

1. SVC (IVC obstruction is rare) 2. Baffle leak

Question 147

The most technically difficult aspect of the arterial switch operation is the __________________

Answer 147

Coronary transfer from the native aortic root to the neo-aortic root

Question 148

Give long term complications post arterial switch operation

Answer 148

1. Coronary artery stenosis - within the first year after arterial switch 2. Aortic dilation and regurgitation 3. Supravalvar PS

Question 149

L-loop TGA, also called _____________________________ Characterized by a. _______ and b. __________________ True or False - L-loop TGA is a cyanotic heart disease

Answer 149

1. Congenitally corrected TGA (ccTGA) 2. AV discordance and ventriculoarterial discordance 3. False - acyanotic Deoxygenated blood goes from the vena cavae to the RA, through the mitral valve to the LV, and then to the PA. Oxygenated blood returns via the pulmonary veins to the LA, through the tricuspid valve to the RV, and then is ejected to the aorta

Question 150

2 procedures in the double switch operation

Answer 150

Atrial switch and Arterial Switch

Question 151

___________________ is a conotruncal anomaly in which both great arteries are completely or nearly completely aligned with the RV

Answer 151

Double outlet RV (DORV)

Question 152

Four common physiologic variations of DORV

Answer 152

1. **VSD physiology**: DORV with large subaortic VSD and no pulmonic stenosis 2. **TOF physiology**: DORV with subaortic VSD and pulmonic stenosis 3. **TGA physiology**: DORV with subpulmonary VSD with or without aortic obstruction 4. **Single ventricle physiology**: DORV with mitral atresia, severely unbalanced AV canal defect, or other cause of significant ventricular hypoplasia

Question 153

This is an uncommon type of congenital heart defect in which a single arterial trunk arises from the heart, giving origin to the coronary arteries, PAs, and systemic arteries, in that order

Answer 153

Truncus arteriosus

Question 154

Thus is a rare CHD lesion in which a membrane separates the LA (sinister) or the RA (dexter) into two compartments.

Answer 154

Cor triatriatum The membrane is proximal to the left atrial appendage, so that the pulmonary veins drain into the proximal chamber and the appendage and mitral valve are in the distal chamber.

Question 155

This consists of a ridge of fibrous or fibromuscular tissue in the LVOT, it is often circumferential extending both onto the septal surface anteriorly and the base of the anterior leaflet of the mitral valve posteriorly. The fibrous tissue may extend onto the aortic valve leaflets contributing to concomitant aortic regurgitation

Answer 155

Subaortic membrane

Question 156

Symptomatic patients with severe LVOT obstruction (maximum gradient ______ mm Hg, mean gradient _____ mm Hg) should be referred for repair

Answer 156

Max gradient > 50 mm Hg, mean gradient > 30 mm Hg Repair is also appropriate for patients with severe obstruction and reduced exertional capacity on exercise testing. Patients with subaortic stenosis and heart failure should also undergo repair, even with moderate gradients (maximum gradient 30 to 50 mm Hg) because the low cardiac output from heart failure may lead to reduced gradients, even if obstruction is severe.

Question 157

____________________ refers to a narrowing in the ascending aorta. Morphologically it is most commonly an hourglass-shaped narrowing but can also occur from a fibrous ridge, or tubular hypoplasia of the proximal ascending aorta _________ gene associated in this condition _____________

Answer 157

Supravalvar aortic stenosis (SVAS) ELN gene, encodes for the elastin protein, on chromosome 7q11.23 Frequently causes stenosis in other proximal arteries - brachiocephalic vessels, branch pulmonary arteries, and ostial coronary arteries Williams syndrome - SVAS affects 65% of patients with WS WS - characteristic dysmorphic facial features (broad forehead, stellate irises, upturned nose, pointed chin; often described as “elfin” facies), hypercalcemia, hypothyroidism, diabetes mellitus, intellectual disability, and often an outgoing (“cocktail party”) personality.

Question 158

This is a localized or tubular narrowing in the aorta or aortic interruption. It is characterized by a generalized arteriopathy with decreased aortic compliance.

Answer 158

Coarctation of the aorta Coarctation is juxtaductal, located at the junction of the distal aortic arch and the descending aorta beyond the origin of the left subclavian artery Associated anomalies - BAV 50-85% Coarctation is common in certain syndromes, including Shone, Turners, Williams-Beuren, Noonan, hypoplastic left heart syndrome (HLHS), and other complex CHD

Question 159

___________________ is the association with coarctation of the aorta with mitral valve disease (parachute or supramitral ring) and multilevel left-sided outflow tract obstruction (subaortic or aortic valve stenosis)

Answer 159

Shone Syndrome

Question 160

CXR finding in CoA where there is erosion of the undersurface of the outer third of a posterior 2nd to 9th rib due to collaterals

Answer 160

Rib notching

Question 161

_______________ refers to the silhouette of aortic dilatation both before (pre-stenotic) and after (post-stenotic) the coarctation site

Answer 161

Figure of 3 sign

Question 162

_________ is routine in CoA diagnosis. Iy is used for quantification of LV mass, evaluating the entire aorta including for arch hypoplasia or Gothic angulation, severity of coarctation, and quantification of collateral flow. It also identifies complications post repair (e.g., aneurysms, false aneurysms, recoarctation, or residual stenosis), presence of aberrant subclavian arteries, and for the morphology and function of the aortic valve.

Answer 162

CMR All adults with coarctation should undergo cross-sectional imaging (usually CMR) at least once.

Question 163

Repair (endovascular or surgical) of coarctation or recoarctation is indicated in hypertensive patients with an invasive peak-to-peak gradient ________________.

Answer 163

>/= 20 mm Hg Coarctation stenting is also considered when feasible for hypertensive patients with a coarctation diameter ≤50% of the aortic diameter at the diaphragm regardless of peak-to-peak gradient and in normotensive coarctation with peak-to-peak gradient ≥20 mm Hg.

Question 164

Types of interrupted aortic arch Interrupted aortic arch is associated with this syndrome ___________

Answer 164

Type A - Interrupted aortic arch occurs distal to the left subclavian artery Type B - between the left carotid artery and the left subclavian artery Type C - between the brachiocephalic trunk and the left carotid artery DiGeorge Syndrome

Question 165

This is an abnormality of aortic arch development which is defined as complete surrounding of the esophagus and trachea by vascular structures Give 2 of the most common forms

Answer 165

Vascular ring Most common forms of vascular ring include **double aortic arch** and **right aortic arch with aberrant left subclavian artery**

Question 166

More common form of anomalous coronary artery from PA

Answer 166

Anomalous left coronary artery from the pulmonary artery (ALCAPA) Myocardial perfusion is via the coronary artery which arises normally from the aorta and is typically quite dilated. Diagnosis should be suspected in infants or young children with ventricular dysfunction or secondary mitral regurgitation

Question 167

Types/forms of single ventricle heart disease

Answer 167

Tricuspid atresia HLHS Double inlet LV Double inlet/outlet RV

Question 168

In patients with single ventricle, this procedure is generally reached in staged palliation, with the ultimate result of all systemic venous return bypassing the heart to flow passively directly to the pulmonary circulation, while the single functional ventricle acts as the systemic pump.

Answer 168

Fontan procedure/palliation

Question 169

Versions of Fontan circulation in adults

Answer 169

Extracardiac Fontan Lateral tunnel Fontan Direct right atrial to PA anastomosis