Cardiology Flashcards
(143 cards)
1
Q
Teenage girl with exercise- induced syncope. She has been diagnosed with long QT syndrome. What is your next step in management ?
A) Calcium channel blocker with high intensity exercise restriction
B) Beta Blocker with high intensity exercise restriction
C) Calcium channel blocker without high intensity exercise restriction
D) Beta blocker without high intensity exercise restriction
A
B)beta blocker with high intensity exercise restriction
2
Q
A child in grade 1 has a history of repaired TAPVR. He has been diagnosed with ADHD. After taking a thorough history and physical, your next step is:
A) ECG
B) ECHO
C) Start a stimulant medication with no further investigation
D) stimulants are contraindicated
A
C) start a stimulant medication with no further investigation
3
Q
3 day old baby with cyanosis, CXR shows large heart and decreased pulmonary markings. Diagnosis?
A) TGA
B) Truncus Arteriosus
C) TOF
D) TAVPR
A
C) TOF
Cyanotic congenital heart disease with decreased pulmonary blood flow
- TOF
- Pulmonary Atresia
- Tricuspid Atresia
- Epstein’s anomaly
4
Q
Below what systolic blood pressure would a 3y.o be considered hypotension ?
A) 64
B) 70
C) 76
D) 84
A
C) 76
Hypotension (systolic) in any ages child is defined via the formula: (age x 2) + 70mmHG
The minimum systolic blood pressure for age are:
- newborns to 1 month old: > 60 mmHg
- 1 month old - 1 year old: > 70 mmHg
- > 1 years old: (age x 2) + 70 mmHg
5
Q
Kid with fhx of uncle who died and has 2/6 SEM worse with standing and has heart palpitations. What do you need to do ?
A) echo
B) ECG
C) Holter
A
A) Echo
HOCM - echo best next step
6
Q
PGE1 being started for a duct dependent lesion in a newborn. Which of the following is Most important to monitor for ?
1) Hypertension
2) Hypoglycemia
3) Hypoventilation
4) Lactic acidosis
A
3) hypoventilation
Complications of prostaglandin E1
FHATS
- Fever + Flushing also Pyloric stenosis
- hypotension
- apnea/ hypoventilation
- Tachycardia
- Seizure + stenosis of pyloric
7
Q
You are seeing a 1 day old newborn, with truncus arteriosus. What are they most likely to develop over the first week of life ?
A) pulmonary edema
B) severe cyanosis
C) shock
D) pulmonary hypertension
A
A) pulmonary edema from CHF
Both ventricles are at systemic pressure and both eject blood into the truncus. When pulmonary vascular resistance is relatively high immediately after birth, pulmonary blood flow may be normal; as pulmonary resistance drops in the first month of life, blood flow to the lungs is greatly increased and heart failure ensues. Truncus arteriosus is a total mixing lesion of pulmonary and systemic venous return. Because of the large volume of pulmonary blood flow, clinical cyanosis is usually mild. If the lesion is left untreated, pulmonary resistance eventually increases, pulmonary blood flow decreases and cyanosis becomes more prominent ( Eisenmenger physiology)
8
Q
10 y/o girl with migratory arthritis for the last 10 days, now affecting left wrist. She is febrile, and has an ESR of 40. Most likely diagnosis is:
A) SLE
B) Rheumatic fever
C) JIA
D) Septic arthritis
A
B) Rheumatic fever
Initial attack:
2 major or 1 major + 2 minor and evidence of recent GAS infection
Recurrent attack:
2 major or 1 major + 2 minor or 3 minor and recent GAS infection
Major
J- joints = migratory arthritis tx ASA
❤️- cardititis = mitral stenosis > aortic stenosis > both
N- nodules subcutaneous on extensor surfaces of arms and occiput of scalp
E- erythema marginatum - evanescent serpanginous circular rash
S- Sydenham’s chorea
Minor
C - elevated CRP
E - elevated ESR
A - arthralgia
prolonged PR interval aka meeting
Fever
9
Q
5 year old with exercise intolerance. On examination he has a slight heave at the left lower sterna border. His first heart sound is normal, the second heart sound is split and quieter. He has a harsh ejection systolic murmur loudest at the left upper sternal border. What is the most likely cause ?
A) mitral stenosis
B) mitral valve prolapse
C) Aortic stenosis
D) pulmonary stenosis
A
D) pulmonary stenosis
10
Q
You are treating a teenage boy with pericarditis. You suspect that his pericarditis might be secondary to SLE. Which of the following is most supportive of this diagnosis?
A) positive RPR
B) positive ANCA
C) positive HLAB27
D) thrombocytosis
A
A. Positive RPR: lupus
Other
ANCA - vasculitis
HLAB27 - ankylosing spondylitis
Thrombocytosis - SLE causes thrombocytopenia
11
Q
What is important to know in order to determine if BP is within normal range ?
A) weight
B) ethnicity
C) height
D) age
A
C) height
12
Q
A 16 year old girl comes to your office. Her BMI is 27 and she has stage 1 hypertension. No protein present in urinalysis. What is the next step in managing her high blood pressure ? A) beta blocker B) calcium channel blocker C) lifestyle D) ACEI
A
C. Lifestyle
13
Q
What is the most common presentation of a 2-day old newborn with cyanotic heart disease ?
A. Bounding/dynamic precordium B) normal pulses and poor perfusion C. Decreased pulses and poor perfusion D. Tachypnea and nasal flaring E. Palpable thrill
A
B. Normal pulses and quiet precordium (could be TGA)
Cyanotic heart disease will present when the duct starts to close and they will probably just be quiet and blue.
- dynamic precordium would be in heart failure, PDA, aortic insufficiency
- decreased pulses would be in outflow tract obstruction
- tachypnea and nasal flaring would be in something that has increased pulmonary flow and not all cyanotic heart disease have increased pulmonary flow ( this could be TAPVD but this is not the most common)
- palpable thrill would only happen with a murmur and not all congenital heart disease have a murmur - mc reason for palpable thrill is restricted VSD
14
Q
Which ECG change is characteristic of rheumatic fever? A) peaked T waves B) prolonged PR interval C) sinus tachycardia D) prolonged QRS
A
B. Prolonged PR interval
15
Q
4 year old healthy kid with slight systolic ejection murmur heard at LLSB and MLSB. There is a variable split S2. Exam is otherwise normal. Cause for murmur ?
A. Benign
B. Bicuspid aortic valve
C. VSD
D. Pulmonary stenosis
A
A. Benign
Murmurs detected after infancy most often represent innocent murmurs but can also be produced by semilunar cable stenosis, AV valve regurgitation, or ASDs. Other congenital heart lesions are unlikely to present at this age unless access to medical care was limited during infancy. Most common is Still’s murmur
16
Q
2 month old baby in SVT ( ECG is rapid, no P waves) - has been feeding poorly last few days. On exam has mild respiratory distress and no peripheral pulses. What is most appropriate next step ?
Or
Baby with poor pulses and looks unwell.
A. Carotid message
B. Adenosine
C. Asynchronous counter shock
D. Digoxin
A
B. Adenosine
Asynchronized shock could cause ventricular fibrillation
17
Q
Teenage girl with recurrent syncope after prolonged standing. Has prodromal symptoms (lightheartedness, etc). What is the most likely diagnosis ?
A. Neurocardiogenic
B. Long QT
C. Postural orthostatic tachycardia syndrome
A
A. Neurocardiogenic - vasovagal syncope
18
Q
Kid with a soft murmur over left upper sternal border, and fixed split S2. Diagnosis ?
A. Pulmonary stenosis
B. Mitral regurgitation
C. ASD
A
C. ASD
This describes the murmur of an ASD - the murmur is from increased pulmonary flow travelling through the RVOT and the delayed pulmonic sound of S2 because of increased flow
19
Q
A 14 year old female with significant family history of sudden cardiac death. Had 2 paternal uncles die of “heart attack “. She has a grade 2/6 SEM worse when standing up and she is hypertensive on exam.
A. Holter
B. Echocardiogram
C. ECG
A
B. Echocardiogram
This is indicative of hypertrophic cardiomyopathy. Not sure why she is hypertensive.
20
Q
Newborn found to have cardiac rhabdomyoma. What syndrome is this a/w ?
A. NF1
B. Tuberous sclerosis
C. WAGR
D. Beckwith Wiedeman
A
B. Tuberous Sclerosis
Dx: 2 major or 1 major + 2 minor or positive genetic testing TSC1 or TSC2
Major
- Hypomelanotic macules (>3, > 5mm)
- Shagreen patch
- Facial angiofibromas ( >3) or fibrous forehead plaque
- Ungual or periungal fibromas (>2)
- Cortical tubers
- Subependymal nodules
- Subependymal giant cell astrocytoma (SEGA)
- Retinal hemartomas
- Cardiac rhabdomyoma
- Pulmonary lymphangioleiomyomatosis (LAM)
- Renal angiomyolipomas
Minor
- Confetti skin lesions (1-2mm hypomelanotic macules)
- Cerebral white matter migration lines
- Dental enamel pits (> 3)
- Gingival/intraoral fibromas (>2)
- Retinal achromatic patch
- Bone cysts
- Nonrenal hemartomas
- Multiple Renal cysts
- Hemartomatous rectal polyps
21
Q
What are screening guidelines for tuberous sclerosis?
A
- Asymptomatic patients = brain MRI q1-3y < 25 (more frequently if SEGA large or growing)
- Renal imaging (US, CT, MRI) q1-3y. Follow angiomyolipoma q1y imaging when size > 4cm
(MRI abdo q1-3y for renal and nonrenal TSC) - Echo q1-3y if cardiac rhabdomyoma until regression/ stabilization
- ECG q3-5y to check for arrhythmias
5.Neurodvlp testing when child starts grade 1 and qvisit - Monitor for signs of increased ICP
- Dental exam q6 months
- BP + GFR + skin + eye exam yearly
22
Q
A 16 year old girl complains of feeling dizzy and palpitations - it occurs within 5 min of standing upright. Her supine blood pressure is 118/70 and her supine heart rate is 84bpm. When she stands up, her blood pressure is 116/68 and her heart rate is 120bpm. What is the diagnosis?
A. Orthostatic hypotension
B. Long QT
C. Postural orthostatic tachycardia syndrome
A
C. Postural orthostatic tachycardia syndrome
Postural tachycardia syndrome = POTS
F> M
Dx: increase in heart rate on tilt table testing or standing
23
Q
There is a 12 year old girl who has had episodes of syncope with exertion while she is playing soccer. She had a sister who died is SIDS last year at 9 months. What ECG finding would be most likely to give you the diagnosis ?
A. Prolonged QTc
B. Wide QRS
C. Prolonged PR
A
Long QT syndrome
Of the arrhythmias a/w sudden death, two are genetic: long QT syndrome and Brugada syndrome
24
Q
Obese teenager with hypertension and a history of two dead uncles from a “cardiac” cause when they were young. He comes to you for the feeling of “skipped beats”. What study will most likely give you the diagnosis ?
A. 24h holter
B. ECG
C. Exercise test
A
A. 24h holter
HCM is a/w arrhythmias, including a-fun and ventricular arrhythmias: ventricular premature beats, non sustained v-tach, sustained v-tach, v- fun. So I guess if he was having symptoms of skipped beats, you want to look for an arrhythmia and 24h holter would be best.
25
Cyanotic infant. CXR shows a large heart and oligemic lung fields ( question describes a CXR, but no actual CXR starched). Which lesion ?
A. TOF
B. Truncus arteriosus
C. TAPVD
D. ASD
A. TOF
Only lesion with decreased pulmonary blood flow and apparently in radiology terms: oligemia= decreased blood flow.
The other lesions have increased pulmonary flow.
- TOF and Tricuspid atresia are cyanotic CHD with decreased pulmonary blood flow
- TRuncus arteriosus - cyanotic heart disease with normal to increased pulmonary blood flow
- TAPVD = fluid overload in lungs
- ASD - a cyanotic heart disease
26
A boy put his finger in an electrical socket and has rhythm below (torsades). He’s in emerge receiving CPR and sats are 100%. What is the next step?
A. Intubate
B. IV/IO epinephrine
C. Synch 1J/kg
D. Defibrillate 2J/kg
D. Defibrillate 2J/kg
I assume if they are doing CPR, there is no pulse
Risk of arrhythmia after electrical injury = 15%, most are benign BUT can result in cardiac arrest (asystole or v-fib). V-fun is mc fatal arrhythmia, occurs in up to 60% of patients when electrical current travels from one hand to the other
Up to date
- prolonged CPR b/c good outcomes even in cases of asystole
- Tx of arrthmias unchanged so in this case, shockable rhythm (polymorphic VT), defibrillation at 2J/kg, magnesium surface helpful in torsades
27
A newborn has cyanosis. His CxR is as follows: slightly boot shaped heart with narrow mediastinum, oligemic lungs
A. TGA
B. tAPVR
C. Truncus arteriosus
D. TOF
D. TOF
28
A teenage boy has a height at 90%, weight at 50%, scoliosis, pectus excavation. What is the cardiac issue most likely to be seen on his echo ?
A. Dilation of aortic root
B. Bicuspid aortic valve
C. Hypertrophic cardiomyopathy
D. Pulmonary stenosis
A. Dilation of aortic root
29
A 5 year girl is referred for assessment of a murmur heard by her family MD. She has a coarse murmur heard in the right subclavicukar area when sitting up, and disappears when she lies down. What is the most likely diagnosis?
A. Still’s murmur
B. Venous him
C. PDA
D. ASD
B. Venous hum
Still’s gets softer when standing up
30
When would endocarditis prophylaxis be required for a patient with a history of endocarditis ?
A. Dental cleaning
B. Umbilical piercing
C. Appendectomy
D. Myringotomy and tunes
A. Dental cleaning
-Appendectomy (no prophylaxis for GI sx unless operating on infected area/sepsis/known wound infection
CPS statement
Abx for dental and other procedures
- give abx in a single dose 30-60min before the procedure or if missed up to 2hours after procedure
- give for dental procedures, reap tract procedures, procedures on infected skin
*prophylaxis no longer recommended for GI or GU Tracy procedures. However for high risk pts who have an established GI or GU tract infection or for those who receive abx to prevent Wound infection or sepsis a/w GI or GU tract procedure, the abx regimen should include an agent active against enterococci, such as ampicillin or vancomycin
Abx= amoxicillin if one allergic then 1st gen cephalosporin (cephalexin), clines, azithro
31
Indications for prophylaxis against infective endocarditis in patients undergoing dental procedures ?
Prophylaxis indicates
HUM - 6RRT
1. Hx of infective endocarditis
2. Unrepaired cyanotic CHD; including palliative shunts and conduits
3. Mechanical/prosthetic cardiac valves (RHD if prosthetic valves or prosthetic material used in valve repair
4. 6R - Repaired CHD with prosthetic material/device, during first 6 months after procedure
5. Repaired CHD with residual defects at the site or adjacent to site of a prosthetic patch/device
6. Transplanted cardiac recipients with valvulopathy
Prophylaxis not indicated:
- ASD, VSD, PDA, MVP, previous Kawasaki, HCM, previous coronary artery bypass graft sx, cardiac pacemakers, bicuspid aortic valves, coarctation of the Aorta, calcifies aortic stenosis, pulmonary stenosis
32
Name the 5 innocent murmurs and characteristics.
1. Still’s/vibratory murmur, age 3-6, SEM at LLSB and apex, vibratory/musical, intensity decreases in upright
2. Venous hum, age 3-6, continuous murmur in subclavicular area R>L, louder when patient upright and changes with jugular vein compression and turning the head
3. Carotid bruit, any age, SEM over neck/carotid area
4. Adolescent ejection murmur, age 8-14, SEM at LUSB, softer in upright
5. Peripheral Pulmonary stenosis, newborn - 6mo, SEM at LUSB/RUSB radiates Scilla and back, harsh, short, high frequency
6. Physiologic pulmonary flow murmur, >3y, SEM at LUSB, softer in upright
33
A 30 month old is found to have a normal s1 and fixed, split s2 on auscultation with a 2/6 murmur at the LUSB. What is his ECG likely to show?
A. Prolonged PR interval
B. Signs of RV overload
C. LBBB
B. Signs of RV overload
- prolonged PR interval = rheumatic fever
- LBBB ( if ASD ostium priming, then will have LAD)
This child likely has ASD fixed s2 with a murmur of RVOT in pulmonic area
EKG shows volume overload of RV: the QRS axis may be normal or exhibit RAD and a minor Right ventricular conduction delay (rSR’ pattern in right precordial leads)
Can have either RBBB or iRBBB a/w ASD
- in RBBB get RSR’ pattern and widened QRS of at least 0.12s (3small boxes) in leads v1-v3
- in incomplete RBBB get RSR’ pattern I n leads V1-V3but no widened QRS
34
8 month old baby with lethargy and poor feeding since yesterday. Today, HR 280 and pulses barely palpable. Weight is 8kg. What do you do next?
A. Defibrillation
B. Adenosine 0.8mg IV
C. Synchronized cardioversion
C. Synchronized cardioversion
C vs B (adenosine vs cardioversion)
Hemodynamically unstable SVT proceed directly to synchronized cardioversion(0.5-1kJ/kg)
- For children with SVT and good perfusion vagal maneuvers can be attempted
- In cases in which SVT is a/w poor perfusion rapidly move to convert the child’s heart rhythm back to sinus rhythm
- adenosine IV rapid push
- if no IV access or adenosine not successful then synchronized cardioversion using 0.5-1.0 joule/kg
* this is probably describing SVT, difficult to palpate pulses in SVT - try adenosine first
VS
* given that child sounds quite unwell, poor perfusion and they don’t mention readily IV access, go straight to synchronized cardioversion. Plus you can’t tell from this question if the child is in SVT or V-tach
35
In fetal alcohol syndrome, what are the most common cardiac abnormalities seen ?
A. Septal defects
B. TGA
C. Bicuspid aortic valve
A. Septal defect
Fetal alcohol syndrome: VSD, ASD, TOF
36
A baby is born post dates and had meconium stained amniotic fluid. After birth he is in respiratory distress. Both pre and post ductal sats are low. He fails a hyperoxia test. What is the diagnosis ?
A. PPHN
B. MAS
C. TGA
A. PPHN ( most neonates present within first 24h of life with signs of respiratory distress
-TGA - would fail hypericum text but usually doesn’t present with respiratory distress
37
Name lesions that cause pre and post- ductal differential:
1. Critical coarctation of the aorta
2. Interrupted aortic arch
3. Critical aortic stenosis
4. PPHN
5. TGA
38
Newborn baby has a murmur. What do you do ?
A. Follow closely
B. Send to family MD
C. Urgent cardio consult
A. Follow closely
Up to date
- in the first few days after birth, most newborns have murmurs that are transient and benign
- usually due to PDA or pulmonary branch stenosis
- pulmonary branch stenosis more likely if murmur has persisted for over 24h when most PDAs have closed
UTD criteria for referral
- Grade > 3 intensity
- holosystolic timing
- maximum intensity at the LUSB
- harsh or blowing quality
- increased intensity with upright position
- diastolic murmur
39
How do you treat a child with hypertrophic cardiomyopathy ?
A. Beta blockers
B. ACE inhibitor
C. Furosemide
A. Beta blocker
40
ECG showing heart block in newborn. What is underlying etiology ?
A. Maternal propranolol
B. Maternal infection
C. Maternal anti-rho antibody
D. Maternal phenytoin
C. Maternal anti-rho
Neonatal SLE
- annular/macular rash of the face, periorbital, trunk, scalp = appears in first 6 weeks of life after exposure to UV light and lasts 3-4 months. Can be present at birth, malar distribution, annular plaques with erythema and scaling
- cytopenias
- hepatitis
- heart block, ranges from 1st degree to 3rd degree, can be detected in fetus as early as 16 weeks GA, usually permanent !!!!
Passive transfer of maternal IgG to fetus. Most cases a/w maternal anti- Rho (SSA) and anti- la (SSB). < 3% dvlp heart block
- increased risk dvlp autoimmune disease
- mom with 15% chance having subsequent child with neonatal lupus after one child born with the condition
- tx with cardiac pacing, excellent prognosis
41
Child with a moderately sized atrial septal defect. What is the most common presentation in an 18 month old with this ?
A. Asymptomatic
B. CHF
C. Exercise intolerance
D. Recurrent respiratory infections
A. Asymptomatic
Nelson
- a child with an ostium sexy fun ASD is most often asymptomatic; The lesion is often discovered inadvertently during PE. Even an extremely large deciding ASD rarely produces clinically evident heart failure in childhood. However subtle failure to thrive may be present and in older children varying degrees of exercise intolerance may be noted. Often the degree of limitation may go unnoticed by the family until after surgical repair, when the child’s growth or activity level increases markedly
42
14 y.o s/p Fontan a few years ago, now diarrhea, and low albumin.
What is condition ?
Protein losing enteropathy
43
Name 3 acute complications post Fontan procedure
1. Elevation in systemic venous pressure
2. Fluid retention
3. Pleural or pericardial effusion
44
What medication can prevent pericarditis post pericardiotomy ?
Colchicine
45
Long term complications of Fontan ?
1. Arrhythmias: SN dysfunction, a flutter/SVT
2. Cyanosis - collaterals, pulmonary AVMs
3. Protein losing enteropathy
4. Plastic bronchitis
5. Thromboembolism- svc/ivc
6. Obstruction of the conduit causing svc/ivc syndrome
7. Liver cirrhosis
8. Ventricular dysfunction
9. Restricted exercises
10. Functional, developmental, psych disorders
11. AV valve regurgitation
12. Kidney disease
13. Lung disease
14. Venous insufficiency
15. Venovenous fistula
46
Endocarditis prophylaxis is indicated for ?
A. TOF
B. MVP with MR
C. Bicuspid Ao valve
A. TOF - unrepaired cyanotic CHD
47
Murmur, LLSB, variable split S2, grade 2/6 murmur
A. PS
B. Bicuspid AV
C. Benign
C. Benign
Normally get split with inspiration and no split with expiration = normal
48
Unstable SVT, what to do ?
A. Amiodarone
B. Adenosine
C. Asynchronous definitely
D. Valsalva
B. Adenosine
49
Infant presents unwell. HR 136, RR50. ECG shows Q-waves in I, aVL, V5 and V6. CXR shows mild pulmonary edema. What is the diagnosis?
A. Anomalous left coronary artery from the pulmonary artery
B. VSD
C. TOF
A. Anomalous left coronary artery from the pulmonary artery
50
Which cardiac defect is most likely in a child with 22q11?
A. TOF
B. VSD
C. Aortic coarctation
A. TOF
51
What do you give a CHF kid?
A. Furosemide
B. Digoxin
A. Furosemide
52
Kid with occasional PVCs, what’s the sports recommendations?
No restriction.
Premature depolarization of a ventricular focus or re-entry. Once structural heart disease and ventricular dysfunction are excluded by ancillary testing, PVCs can be considered benign.
53
What are the indications for further investigations for PVCs and what is the treatment ?
1. >2 PVCs in a row
2. Multiform PVCs
3. Increased ventricular ectopic activity with exercise
4. R on T phenomenon
5. Extreme frequency of beats ie; > 20% of total beats on holter monitoring
6. Underlying heart disease or hx of heart surgery
Treatment
1. Correct underlying abnormality
2. Lidocaine bolus and drip
3 amiodarone for refractory, v disfunction or HD compromise
54
Endocarditis prophylaxis?
A. TOF
B. VSD closed with residual leak
A. TOF
Controversial - why not b. Maybe
55
What is treatment for infective endocarditis? And alternatives for allergic patients ?
A. Amoxicillin
| If allergic cephalexin, clindamycin, azithromycin or clarithromycin
56
What’s the most common cardiac lesion in an IDM?
A. HCM
B. ASD
C. VSD
A. HCM
57
ECG tracing showing bradycardia/heart block. What are you most likely to find on maternal history?
A. Parvovirus
B. SLE
A newborn is found to have a rash and anemia. An ECG is shown with what looks like complete heart block (dissociation btw p waves and QRS; QRS rate = 6 blocks apart: HR 50). What is the most likely illness that would be found in the mother ?
A. SLE
B. Hyperthyroidism
C. Addison
SLE
58
Girl with high symptoms with high BMI and hypertension. Which of the following tests is most likely to reveal an underlying sequel of her disease ?
A. Echo
B. ECG
Echo - looking for LVH or for pulmonary from OSA
59
Boy with TAPVD. Want tos tart ADHD Med. What do you do ?
A. History and physical and consult with cardiology
B. ECG
C. Cardiology consult
D. Echo
A. History and physical and consult with cardiologist
For ADHD pts with known heart disease who are followed by a cardiologist the physician with expertise in ADHD likely remains the appropriate individual to evaluate benefit and risk and make a recommendation for medication therapy, because there is little evidence that taking medication further increases the risk of sudden death.
60
What was the reason for revising guidelines for cardiac prophylaxis ?
A. Cost effective
B. Infections were more likely from exposures from everyday life and not that much more from dental procedures
B. Infections more likely from every day infections
61
Soft second heart sound with click. Left parasternal heave. Systolic murmur at left upper sternal border. What is the defect ?
Pulmonary stenosis
Most pts with PS are asymptomatic and typically do not have any cardiac physical findings. Cyanosis may be seen in infants with a significant right to left shunt due to RVOTO
62
6 week old with pansystolic murmur, increasing respiratory distress and liver edge down. CXR shows increased pulmonary markings. Which medication would you consider to help his symptoms ?
A. Propranolol
B. Furosemide
C. Digoxin
D. Adenosine
3 week old baby with tachypnea RR70, chest crackles, diaphoresis with feeding. Grade III/IV murmur pansystolic, liver at 5cm below costal margin. Treatment:
A. Digoxin
B. Furosemide
C. Prostaglandin
Furosemide
Baby likely has A large VSD leading to pulmonary edema and CHF. Treat fluid overload with Ladin
```
Describing CHF from VSD
3 causes of pansystolic murmur:
1. VSD
2. Tricuspid regurgitation
3. Mitral regurgitation
```
63
Baby is refusing to feed since this morning. He is pale and lethargic over the last 24h. HR 300, RR 70, weak pulses. What did you do? Baby weight is 8 kg ?
A. Synchronized 4J cardioversion
B. Synchronized 8J cardioversion
C. Asynchronized 4J cardioversion
D. Asynchronized 8J cardioversion
A. Synchronized 4J cardioversion
(0.5 J/kg)
Tricky question because you could start with either 0.5-1J/kg and if not effective increase to 2J/kg
64
5 day old with tachycardia, tachypnea, cyanosis and left Sa02 is 91% and right Sa02 is 98%. Best next step is:
A. Start PGE1
B. Give indomethecin
C. Intubate
D. CXR
Start PGE1
This is likely a child with interrupted aortic arch arch, critical aortic stenosis etc which is a duct dependent systemic circulation lesion.
Exam tip - the only time you don’t give prostin is in TAPVR (worsens fluid overload).
65
Newborn diagnosed with interrupted aortic arch, what to start ?
A. Dopamine
B. Prostaglandin
C. Nitric oxide
D. Indomethacin
B. Prostaglandin
Those infants with just narrowing of the transverse arch may respond to inotrooes and diuretics. With severe arch narrowing, PGE1 is extremely useful for dilating the duct is arteriosus and returning adequate flow to the lower body. This will allow the infant to stabilize for surgery
66
In the most common long QT syndrome (Romano- ward), you ask about:
A. Deafness
B. Family history
B. Family history
67
In a patient with Romano- Ward syndrome, what would suggest the diagnosis:
A. Presence of cafe au lait spots
B. Congenital defects
C. Sensorineural hearing loss
D. Family members have it
D. Family members have it
68
Long QT associated with ?
A. Hypokalemia
B. Hypercalcemia
C. Clarithromycin
D. Digoxin
A. Hypokalemia but also clarithromycin
Etiologies for prolonged QT include:
1. TCA overdose
2. Hypocalcemia
3. Hypomagnesemia
4. Hypokalemia
5. Type 1a and III antiarrhythmics (Ia = quinidine, procainamide; III = amiodarone, sotalol)
6. Starvation with electrolyte abnormalities
7. CNS insult
8. Non- sedating antihistamines
9. Azithromycin
10. Liquid protein diet
69
Infant with right arm saturation of 90% and left leg saturation of 70%. Patient tachypneic, RR 70, no distress, no distress. Diagnosis ?
A. Coarctation of the aorta
B. Truncus arteriosus
C. TGA
D. TOF
A. Coarctationof the aorta
- TGA- both pre and post ductal sats low and pre can be lower than post
- TRuncus arteriosus - one common great vessel - sats are the same as they don’t have a duct
Preductal saturation is normal, where post- ductal saturation is low; patient is also tachypneic for age. This is suggestive of a duct dependent lesion presenting as the PDA is closing.
Duct dependent lesions for systemic circulation include:
1. HLHS
2. Coarctation of the aorta
3. Interrupted aortic arch
4. Critical aortic stenosis
70
PAtients with ASDs have a fixed split S2. This is due to :
A. Pulmonary hypertension
B. Abnormal pulmonary valve
C. Pooling of blood in the pulmonary vasculature
D. Prolonged right ventricular ejection
D. Prolonged right ventricle ejection
71
12h old newborn has a saturation of 80%. Increases to 85% with 100% 02. Mild tachypnea RR 65. CXR has no abnormalities. What next initial management should be done ?
A. Intubate and Ventilate
B. Prostaglandins
C. Antibiotics
Controversial A vs B.
A. Intubate and ventilate
Stabilize baby then PGE1
Or
B. Protaglandins
? Maybe ventilating will have no further effect on oxygenation - I would go with this one !!
Hyperoxia test - useful in distinguishing cardiac from pulmonary causes of cyanosis.
Hypoxia in many heart lesions is profound and constant, whereas in resp disorders and in PPHN, arterial oxygen tension often varies with time or changes in ventilator management.
72
12h old newborn with cyanosis, sats 80% increased to 85% on 100% 02. CXR normal pulmonary vasculature and no other anomalies. Most likely diagnosis?
A. HLHS
B. TOF
C. TGA
D. Tricuspid atresia
Controversial between C.TGA AND D. Tricuspid atresia
Answer: D. Tricuspid atresia
A. HLHS = increased pulmonary vasculature due to LVOTO and cardiomegaly on CXR
B. TOF = decreased pulmonary vasculature, presents with enlarged boot shaped heart
C. TGA = increased pulmonary vasculature, CXR egg on a string but can have a normal CXR
D. Tricuspid atresia = decreased pulmonary blood flow and LVH + RAE on CXR
Decreased pulmonary flow
- TOF
- Pulmonary atresia it severe stenosis
- tricuspid atresia
- Epstein anomaly
Increased pulmonary flow
- TGA or can be normal
- TAPVR
- TRuncus arteriosus
73
Infant of a diabetic mother with non- obstructive hypertrophic cardiomyopathy, child is macrosomic but otherwise asymptomatic, glucose fine, with no signs of heart failure; how will you manage the cardiomyopathy?
A. Treat with steroids
B. Treat with digitalis
C. Treat with diuretics
D. Will resolve without treatment
D. Will resolve without treatment
If symptomatic treat with beta blocker
74
What is the natural history of an infant with hypertrophic cardiomyopathy if the mom had gestational diabetes ?
A. Long term complications, requires transplantation
B. Improves with digoxin and diuretics
C. Spontaneous resolution
D. Improves with beta blockers
C. Spontaneous resolution
Infants often asymptomatic but 5-10% have resp distress or signs of poor cardiac output or heart failure. The chest radiograph may show cardiomegaly
75
At a regular office visit, a 7 y.o girl is noted to have a continuous murmur in the right subclavicular area that is louder when upright. She is well and has no other contributory medical history.
A. Still’s mumur
B. PDA
C. Venous hum
C. Venous hum
76
Infant presents pale, poorly perfused, big liver edge, looking like crap with HR of 260 after a few days of not acting right. Diagnosis ?
A. Cardiomyopathy
B. SVT
C. Metabolic disorder
B. SVT
77
5 year old kid comes in with pneumonia. You discover a loud S1, fixed s2 and a murmur at the LUSB. Most likely diagnosis?
A. ASD
B. PDA
C. PS
A. ASD
78
Child on resperidone for Tourette syndrome has frequent sun opal episodes with exertion. What is the cause?
A. Hypoglycaemia
B. Prolonged QT
B. Prolonged QT
79
Infant with a large VSD. The murmur cannot be heard. What is the cause ?
A. VSD has closed
B. There is increased pulmonary outflow obstruction
C. Pulmonary arterial pressures have increased
C. Pulmonary arterial pressures have increased
If your VSD is very large you will have lots of L to R shunting which will
Lead to even higher pulmonary pressures. This can lead to pulmonary HTN which will increase shunting from R to L and you will basically have an open chamber with no shunting as your pulmonary pressures will be close to your systemic pressures.
80
What is the most common physics exam finding in congenital cyanotic heart disease ?
Cyanosis
81
Well newborn is examined on day 1 of life, feeding well. Found to have normal exam except for a grade 2/6 systolic murmur at the left sternal border. Normal pulses. What do you do ?
A. CXR
B. EKG
C. Echo
D. Nothing for now, observe
D. Nothing for now - observe
Likely PPS - peripheral pulmonary branch stenosis
In the first few days after birth, most newborns have murmurs that are transient and benign. They usually are caused by a PDA or pulmonary branch stenosis. PPS is more likely if the murmur persists after 24h when most PDAs have closed.
82
A 3 day old is tachypneic, cyanosis despite 100% 02. Bilateral crackles on exam with weak peripheral pulses and no heart murmur. What is the diagnosis ?
A. HLHS
B. Sepsis
C. AV fistula
A. HLHS
83
You are seeing a teen with a history of recurrent syncopal episodes. What is the best screen for long QT syndrome ?
```
A. Exercise ECG
B. Holter
C. Echo
D. ECG
E. Electrolytes
```
D. ECG - ultimately all of these will
| Probably be done but ECG will obviously show you if there is a long QT or not
84
A 10 y.o underwent valvotomy for aortic stenosis 7 years ago. He developed varicella 11 days ago and now presents with a 6 day history of lethargy, anorexia, weight loss, and low grade fever. Findings on PE include loud, high pitched diastolic murmur. Of the following, the most likely reason for current findings:
A. CHF
B. IE
C. Myocarditis
D. Pericarditis
B. Infective cardititis
High pitched diastolic murmur suggests aortic regurgitation/incompetence in a child who has an abnormal aortic valve. He likely has a secondary bacterial infection after varicella with group A strep ( a more unusual bug).
Most common bugs
1. Strep viridans
2. Staph aureus
3. Enterococcus (Group D strep)
85
Describe 4 stages of ECG changes in pericarditis.
Stage 1: widespread ST elevation and PR depression with reciprocal changes in aVR - occurs during first 2 weeks
Stage 2: normalization of ST changes; generalized T wave flattening (1-3 weeks)
Stage 3: Flattened T waves become inverted (3 to several weeks)
Stage 4: ECG returns to normal (several weeks onwards)
86
A 14 year old is being medically evaluated for Highschool football team. History reveals that he had a brief episode of syncope while playing basketball during the previous summer. PE normal. Which of the following studies would be most useful ?
A. ECG
B. Echo
C. Holter
D. Treadmill
A. ECG - would be your first test
87
A 6 year old child is vomiting and has abdominal pain. 3 weeks ago she had repair of a secundum ASD. Findings include listlessness and pallor. Temp 40, HR 140, Bap 85/52, RR 36/min. Mild tenderness to deep palpation of the abdomen and decreased intensity of femoral pulse as she inhaled. The most appropriate initial study ?
A. Abdominal U/S
B. CBC and blood culture
C. Echocardiogram
D. ECG
C. Echo
This is post pericardiotomy syndrome with malaise, abdominal pain, vomiting, pallor with fever and tachycardia. This is pulsus paradoxes because of decreased femoral pulses and she is unstable. Need echo to decide about pericardiocentesis.
Complications of ASD repair occur in 25-30% and include: pericardial effusions, pleural effusions, arrhythmias, bleeding, pneumothorax, wound infection.
- pericardial effusion/tamponade can result in decreased cardiac output (hypotension, elevated JVP, pallor, lethargy), muted heart sounds and pulsus paradoxus ( BP drops during inspiration due to RV compressing LV and decreasing preload and cardiac output ).
88
Concerning complete AV block, all are true except ?
A. May be a cause of syncopal episodes
B. Ventricular rate of 30-60 bt/min
C. If you hear a systolic murmur then it is associated with congenital heart lesion
D. Diagnosis is confirmed by ECG
E. May be present in infants born to mothers with SLE
C. If you hear a systolic murmur then it is associated with a congenital heart lesion - could be an acquired cause of a murmur
89
3 day infant cyanosis with crying, investigation
```
A. ECG
B. CXR
C. ABG
D. Blood culture
E. Echo
```
E. Echo - diagnostic - sounds like a tet spell
90
An infant is in shock with paroxysmal supraventricular tachycardia. You would give:
```
A. A bag of ice to face
B. Synchronous DC cardioversion
C. Asynchronous cardioversion
D. Verapamil
E. Digoxin
```
B. Synchronous DC cardioversion as they are unstable at 0.5-1J/kg and increase to 2J/kg if not effective
91
Newborn baby with cyanotic congenital heart disease. Most consistent physical exam finding ?
```
A. Bounding/dynamic precordium
B. Normal pulses and quiet precordium
C. Decreased pulses and poor perfusion
D. Tachypnea and nasal flaring
E. Palpable thrill
```
B. Normal pulses and quiet precordium
A. Bounding/dynamic precordium - suggests increased volume from L to R shunt ie PDA, sepsis, fever
B. Normal pulses and quiet precordium
C. Decreased pulses and poor perfusion - coarctation
D. Tachypnea and nasal flaring - resp distress suggests resp problem but TAPVD with obstruction, HLHS, critical AS and severe coA can also present with resp distress
E. Palpable thrill- palpable murmur - a pathological murmur is audible in most forms of cyanotic CHD but a thrill is severe and not consistently felt ( may be felt with severe aortic or pulmonary stenosis
92
3 day old infant with congenital heart disease whose cyanosis is aggravated by crying. Most likely diagnosis ?
```
A. TGA
B. VSD
C. ASD
D. PDA
E. PS
```
Controversial TGA vs PS
E. Pulmonary stenosis - sounds like a tet spell. PS is one of the features of TOF
But TGA with large VSD - cyanosis may be mild initially although it is more apparent with stress or crying
93
A 3 day old infant develops poor perfusion, diminished peripheral pulses, and decreased urine output. What medication would you administer ?
```
A. Atropine
B. Morphine
C. Bicarbonate
D. Furosemide
E. Prostaglandins
```
E. Prostaglandins - ductal dependent lesion for systemic circulation therefore start prostaglandins
94
SVT in neonates is most commonly associated with:
```
A. Hypocalcemia
B. Hyperthyroidism
C. Electrolyte disturbances
D. Ventricular septal defect
E. Structurally normal heart
```
E. Structurally normal heart
The majority of patients with SVT have structurally normal hearts. The 2 Mx forms of SVT in children are:
1. atrioventricular reentrant tachycardia (AVRT), including the WPW syndrome
2. Atrioventricular nodal reentrant tachycardia (AVNRT)
95
An ECG is shown. Left axis deviation, increased forces, ST- T changes with T- wave inversion
A. LVH
B. RVH
C. Heart block
D. Wolf- Parkinson- White
A. LVH - left axis deviation, R in V5/6 + S in V1/V2 > 35mm (or greater than limit for patient’s age )
Rest
B. RVH - right axis deviation, v1 increased R/S ratio (greater than upper limit for age), V6 R/S <1
C. Heart block - atrial rate completely dissociated from ventricle
D. WPW - delta wave, shortened PR
96
A 10 year old who underwent recent dental surgery now presents with fever, arthralgias, splenomegaly, and lesions on the hands and feet
```
A. Septic emboli
B. Subacute endocarditis
C. Acute rheumatic fever
D. Juvenile rheumatoid arthritis
E. Coxsackie virus
```
B. Subacute endocarditis
He has multiple minor features of the dukes criteria
97
What are features of Duke criteria for infective endocarditis?
- Definite IE = pathological criteria or clinical criteria ( 2 major OR 1 major + 3 minor OR 5 minor)
- possible IE = 1 major + 1 minor OR 3 minor
- rejected IE = firm alternate dx, resolution with abx for <4 days, no pathologic evidence, does not meet above criteria
Major criteria
1. Blood culture x2 positive with typical microorganisms for IE ( strep viridans, strep gallolyticus, HACEK, staph aureus, enterococcus) or 1X culture with coxiella burnetti or anti phase 1 IgG antibody titre > 1:800
2. Evidence of endocardial involvement on echo
Minor
1. Predisposing heart condition or IV drug use
2. Fever > 38
3. Vascular phenomenon: major arterial emboli, septic pulmonary infarcts, mycotoxins aneurysms, IVH, conjunctival hemorrhage, Janeway lesion
4. Immunologic phenomenon: glomerulonephritis, Osler nodes, Roth spots, rheumatoid factor
5. Microbiologic evidence: positive blood culture that does not meet major criteria or serologic evidence of active infection with organism consistent with IE
98
What are the antibiotic regiments for dental procedure prophylaxis ? If unable to take oral meds ? If allergic ? If allergic and unable to take oral meds ?
PO Amoxicillin
IV ampicillin or cefazolin/ceftriaxone
Allergic oral - cephalexin, Clindamycin, azithromycin/clarithromycin
- allergic and IV needed - cefazolin/ceftriaxone, clindamycin
99
A full term infant is diagnosed with meconium aspiration syndrome. He is desaturation despite 100% oxygen. An echocardiogram shows normal cardiac anatomy and R to L shunting at the ductus and atrial level. Which of the following interventions may be effective?
A. Dopamine
B. Indimethacin
C. Nitric oxide
D. Prostaglandins
C. Nitric oxide - this is PPHN
Selective pulmonary vasodilator used in persistent high pulmonary vascular pressure and resultant Right to left shunting of blood through the foramen ovals, ductus arteriosus and intraouonary channels. Used in infants >/= to 35 weeks GA. Usually started when OI > 20-25 or when the Pa02 remains < 100mmHg, despite optimal ventilation with 100% oxygen.
100
What is the treatment for primary pulmonary hypertension? Describe steps in management
1. Tx underlying condition
2. Supplemental 02
3. Acute - short term iNO
4. Sildenafil and Bosentan
5. IV or SC prostacyclin
6. Anticoagulation
7. Lung transplant
101
Name 4 etiologies of primary pulmonary hypertension
1. Idiopathic or familial disease (55%)
2. Pulmonary HTN 2nd to CHD (35%)
3. Chronic respiratory disorders (15%)
4. Sickle cell anemia and other hemolytic anemia’s
102
What are findings in CXR of primary pulmonary hypertension ?
1. Prominent pulmonary artery and right ventricle
| 2. Pulmonary vascularity in hilar areas may be prominent, decreased in peripheral lung fields
103
What is the gold standard for diagnosis, assessment of severity and treatment of pulmonary hypertension ?
Cardiac catherization
104
What is the management of vasovagal syncope ?
1. Reassurance and education
2. Cross legs if prodromal signs
3. Encourage fluids and salt
4. Second step is treatment with fludrocortisone
5. Patients with prominent hypotension tx with midodrine - alpha agonist
105
Which of the following pulse profiles matches the diagnosis given ? Give causes of each type of pulse
A. Pulsus alternans - constrictive pericarditis
B. Atrial fibrillation - hypothyroidism
C. Pulsus bigeminus - digoxin toxicity
D. Dicrotic pulse - mitral stenosis
C. Pulsus bigeminus - digoxin toxicity
Others should be:
A. Pulsus alternans - pericardial effusion not constrictive periscarditis
B. Atrial fibrillation occurs in hyperthyroidism not hypothyroidism
C. Dicrotic pulse - low CO ie: heart failure, shock
Pulsus alternans - variation in amplitude of the systolic arterial pressure with every other beat.
1. Associated with severe left ventricular systolic dysfunction and
2. pericardial effusions
Atrial fibrillation - causes include ischemic heart disease, hypertension, valvular heart disease (MS/MR), acute infections, electrolyte imbalances (hypokalemia, hypomg), thyrotoxicosis ie; hyperthyroidism, drugs (sympathomimetics), PE, pericardial disease, pre-excitation syndromes, HOCM, pheochromocytoma
Pulses bigeminus- every other beat is a PVC. Causes include anxiety, sympathomimetics, beta agonists, excess caffeine, hypokalemia, hypomagnesemia, digoxin toxicity and myocardial ischemia
Dicrotic pulse- the dicrotic pulse is an abnormal carotid pulse found in conjunction with certain conditions characterized by low cardiac output. It is distinguished by two palpable pulsations, the second of which is diastolic and immediately follows s2. Tends to occur in pts with decreased systemic arterial pressure and low cardiac output. May also present in heart failure, hypovolemic shock, cardiac tampanade, elevated SVR
106
Picture of narrow complex tachycardia, no palpable pulse with poor perfusion:
A. Carotid massage
B. Asynchronous cardioversion
C. Adenosine
D. Verapamil
C. Adenosine - SVT but unstable if synchronized cardioversion in question pick that one instead
107
What is most characteristic of a Still’s murmur ?
A. Vibratory murmur
B. Increased with sitting
C. Often radiates to axilla
A. Vibratory murmur
Innocent Still’s murmur- systolic murmur with maximum intensity at the LLSB and apex and has minimal radiation. The murmur has characteristic vibratory or musical quality. Is quieter in sitting position. Louder in hyper dynamic states ie: fever, anxiety and usually is grade 1-2 intensity. Usually resolves by adolescence.
B- it decreases with sitting
C- has no radiation
108
Baby boy born at term with no problems. You’re seeing him at 6 weeks now, mom had noticed he is not feeding well. You note he has been gaining weight poorly. Exam is remarkable for a harsh pansystolic murmur at the LSB, radiating to the right.
A) what is the diagnosis? (1point)
B) explain the reason he is presenting with these symptoms now ? (2 points)
A) VSD
| B) change in the pulmonary and systemic pressure. L to R shunt and congestive heart failure
109
Newborn baby, a few hours old, is cyanotic. You suspect cyanotic heart disease but you don’t have access to an echo at the community hospital you’re working at.
A). Aside from an ECG and CXR, what are two tests that you do to confirm cyanotic heart disease?
B) what treatment would you start while awaiting the transport team
A)- 4 limb BP
- pre and post ductal sats
- hyperoxia test
B) prostaglandin infusion and possibly intubation in cases of apnea
110
Three week old infant presents lethargic and pale, with a two- day history of poor feeding. HR 210, BP 44/35. The ECG shows a narrow complex tachycardia and you make a diagnosis of supraventricular tachycardia.
A) what are two things on the ECG, apart from the narrow complex, that would be consistent with SVT?
B) now the baby has a blood pressure of 40/20 and pulses are thready. You have given one dose of adenosine without effect. What are two other pharmacological measures you could use to treat the SVT ?
A) ECG findings if SVT
- p waves cannot be discerned, they are usually buried in the QRS complex
- rate that is usually > 220 in infants and > 180 in children
- regular rhythm ( no variation in the RR interval)
- the QRS complex is narrow (<80 msec)
B) two other meds
- Amiodarone
- procainamide
111
A 14 year old boy has hypertension. He is at the 99th percentile for height and weight.
A) What are the three investigations you need to do to assess for effects of his hypertension, and what would you be testing for ? ( 3 points)
B) if this boy wants to play hockey, what finding from an investigation would prevent him from doing so ?
A)
1. Echo, ECG — LVH
2. Fundoscopy — retinopathy
3. U/A, BUN/Cr — nephropathy
B) LVH
112
Teenage boy with chest pain. List 5 things in the history that would make you think this is cardiac in origin ?
1. Retrosternal chest pain radiating to left arm or jaw, a/w vomiting, diaphoresis, dyspnea- classic angina
2. Pain worse with exertion and improves with rest - MI, coronary anomalies, cardiomyopathy
3. Pain associated with palpitations, syncope, dizziness, SOB
4. Presence of risk factors: previous Kawasaki, familial hypercholesterolemia, use of cocaine
5. Family history of sudden death, HOCM
6. Pain is poorly localized and not reproducible
7. Severe, tearing pain often radiating to back - aortic dissection
8. Sharp, retrosternal pain exacerbated by lying down and relieved by leaning forward, sometimes radiating to left shoulder, often a/w fever - pericarditis
9. Pain precipitated by cocaine, amphetamines, bath salts, synthetic canna I pods, marijuana or other vasoactive drugs - variant angina
113
A 12 year old girl comes to your office complaining of worsening shortness of breath with exertion. You find she has a hemodynamically significant ASD.
A). List 3 auscultory findings of an ASD
B). List 2 long- term complications of an unrepaired ASD
A. 3x auscultory findings
1. SEM best heard at LUSB
2. Wide Fixed split S2
3. Diastolic rumble
4. Right ventricular heave
5. First heart sound can be split
B. 2x complications
1. Atrial Arrhythmias from atrial enlargement - presents as palpitations
2. Pulmonary hypertension - Eisenmenger syndrome
3. Heart failure with moderate to large ASDs (fluid retention, hepatimegaly, elevated JVP) - later in life
4. Paradoxical embolization of clot from venous system or RA via R to L shunting into the arterial system
114
You see the following ECG. It’s an ECG with wide QRS and delta waves.
A. What is the major abnormality on the ECG?
B. What condition is this most commonly associated with ?
C. What rhythm is this patient at risk for?
D. What is the definitive treatment ?
A. Major ECG abnormality
- wide QRS with delta waves and short PR interval
B. What condition?
- WPW
C. What rhythm at risk for?
- SVT
- a-fib with rapid AV conduction degenerating to V- fib
D. Definitive treatment
- catheter ablation
115
Table given with medications write “increase”, “decrease”, or “nine” for effect of the following on a) contractility and b) PVR and c) B2 vasodilation. Also give doses of meds and side effects and what receptors they act on
- epinephrine low, high dose
- dopamine low, Med, high dose
- dobutamine
- milrinone
- norepinephrine
- isoproterenol
- phenylephrine
A. Contractility B. SVR C. B2
Drugs
1. epinephrine
-low dose (0.05mcg/kg/min) = B1, B2 > a1, s/e increased myocardial 02 consumption. Increases HR and contractility
A. Increased B. None C. ++
-high dose (0.5 mcg/kg/min) = a1 > B1, B2, potent vasoconstrictor, s/e decreased renal perfusion, increased risk of cardiac arrhythmias
A. Increased B. Increased C. +
2. Dopamine
- low dose 0.5-2 mcg/kg/min, B1 + Dopa
A. Increase B. None C. None
- med dose 5-10mcg/kg/min, B1 > a1 + Dopa, s/e increased arrhythmias
A. Increase B increase C. None
- high dose 20mcg/kg/min, a1 > B1
A. None B increase C none
3. Dobutamine 1- 10 mcg/kg/min, B1>B2>a1, increased inotropy and vasodilation
A. Increased B. Decreased C. ++
4. Milrinone PD3 inhibitor, inotrope and vasodilator
A. Increase B decrease C ? ++
5. Norepinephrine 0.05- 1.5mcg/kg/min, a1>B1, potent vasoconstrictor
A. None B. Increase C. None
6. Isoproterenol, acts on B1 + B2,
A. Increase B. Decreased C ++
7. Phenylephrine 0.5-2 mcg/kg/min, potent vasoconstriction
A. None B increase C. None
116
7 year girl with “confirmed” hypertension (didn’t say how it was confirmed). Has had normal CBC, urea, creatinine, electrolytes. Name 4 investigations you would do at this point.
Initial lab evaluation in all children with persistent HTN is directed at
a) determining etiology of HTN
B) identify other CVD risk factors
C) detect end-organ damage
1. Renal u/s with Doppler
2. Plasma and urine catecholamines
3. Echo to look for coarctation, LVH
4. TSH, T4
5. Serum renin, aldosterone
6. 24h ambulatory blood pressure monitoring(unless confirmed means already done)
7. Serum/urine drug screen ( probably not high yield in a 7 year old)
8. Ophtho exam - looking for end organ damage
9. Urinalysis/ urine dipstick
10. Lipid profile (HDL & cholesterol )
All patients
1. Urinalysis
2. Chem panel: lytes, BUN, Cr
3. lipid profile (HDL + cholesterol)
4. Renal Doppler U/S in those < 6y or those with abnormal U/A or renal function
```
Obese = BMI > 95%
In addition to above
1. Hemoglobin A1C - screen for diabetes
2. LFTs - screen for fatty liver
3. Fasting lipid profile
4. Echo to be done at time of pharmacological treatment of HTN to assess LVH. Also can rule out coA
```
Optional tests based on hx, PE, initial investigations
1. Fasting serum glucose
2. TSH, T4 - hyperthyroidism
3. CBC - to look for anemia that may reflect CKD, vascular is or polycythemia
4. Drug screen + ethanol level
5. Sleep study - OSA
6. Mineralcorticoids, aldosterone, renin - hyperaldosteronism
7. Plasma and urine catecholamines for pheochromocytoma and Neuroblastoma
117
A term baby is born to a mother with maternal lupus. He is noted to have congenital heart block.
A. Name the two most common antibodies in congenital heart block
B. Name 3 other manifestations of neonatal lupus
A. Anti- Rho (SSA) and Anti- La (SSB)
B. 3x Other manifestations
1. Skin/Rash - round erythematous, papulosquamous lesions with central clearing, annular erythema and fine scale - typically involves the face/scalp, periorbital areas. Exacerbated by UV light, becomes apparent during first three postnatal months
2. Liver - transaminitis, cholestasis, hepatosplenomegaly
- mc presentations are
a) direct hyperbolic with mild or no transaminitis in the first few weeks after birth or
b) mild transaminitis occurring at 2-3 months
3. Heme - cytopenias (anemia and thrombocytopenia mc)
4. Autoimmune thyroid disease - hypo or hyperthyroidism
5. Pulmonary - pneumonitis, pulmonary capillarity is (rare)
6. Renal - glomerulonephritis, nephrotic syndrome (rare)
7. Cardiac - structural heart disease (rare), ASD/VSD/AVSD/ PDA/PFO, PS, TGA, also endocardial fibroelastosis, myocarditis
118
An 11 month old girl comes in lethargic, febrile with HR 180, bounding pulses, warm feet.
A. What is her cardiac output likely to be compared to normal?
B. What is her systemic vascular resistance likely to be compared to normal
C. How would you classify her physiologic state?
D. Write your initial IV fluid order for this girl
Septic shock - in the early stages, you have warm shock - cardiac output increases in an attempt to maintain adequate oxygen delivery and meet the greater metabolic demands of the organs and tissues.
A. Cardiac output - increased
B. SVR - decreases
C. Warm shock (early stage of septic shock) if they give a BP, could say compensated based uncompensated
D. Rapid IV bolus of 20ml/kg of 0.9% normal saline; blouses should be repeated up to 60-80ml/kg
Norepinephrine is your first choice vasopressor for warm shock....this makes sense because it is a potent vasoconstrictor with no effect on cardiac contractility
119
12 year old child is referred to you for assessment of a murmur.
A. What is one clinical characteristic of the murmur that would be very reassuring to you in providing that the murmur is benign?
B. Name three innocent murmurs
A. Clinical characteristic of benign murmurs
1. Grade <2 intensity
2. Softer intensity when the patient is sitting compared to when the patient is supine
3. Short systolic duration ( not holosystolic or diastolic)
4. Minimal radiation - the murmur is located in a limited region of the precordium
5. Musical or vibratory quality
B. Types of innocent murmurs
1. Still’s/vibratory murmur
2. Venous hum
3. Physiologic pulmonary (flow)systolic ejection murmur,
4. neonatal peripheral pulmonary stenosis/benign pulmonary branch stenosis
5. Supraclavicular arterial bruit
6. Carotid bruit
7. Adolescent ejection murmur
120
6 month old with congestive heart failure secondary to congenital heart disease. Not gaining weight despite attempts to provide adequate calories. List 4 reasons why this child may not be gaining weight.
1. Increased metabolic demand due to CHF
2. Inadequate intake as child may tire with feeding (this despite attempts to provide adequate calories, the child falls asleep or tires during feeds)
3. Child is aspirating (if tachypneic secondary to CHF) - trouble coordinating feeding and breathing
4. Use of diuretics to treat CHF
5. Poor parental- child interactions ie; due to sick baby
6. The congenital heart disease is associated with a genetic syndrome ie; Turner’s, trisomy 21, Williams
utd
Poor feeding is one of the most important stressors that an infant might experience. Although the history is often nonspecific, poor feeding and weight gain are common findings in an infant with significant cardiac disease and limited reserve.
Hx often reveals:
1. Lethargy and tiring with early stopping of feeds
2. Respiratory distress with tachypnea, retractions, and/or wheezing
3. Trouble coordinating feeding and breathing
4. Irritability
5. Sweating
failure to thrive second to increased metabolic demand and inadequate intake.
121
A 4 month old presents with hepatomegaly, pallor and sweating while feeding. Sa02 96% and working hard with feeds. What are 3 primary cardiac causes that could account for congestive heart failure ?
1. Increased preload ie; L to R shunts or valvular insufficiency = VSD, PDA, MR
2. Increased after load = coA, AS
3. Cardiomyopathy ( cardiac muscle has compromised intrinsic contractility) = myocarditis, cardiomyopathy
4. Dysrhythmia
Most common cause of CHF in infants < 6 months = volume overload lesions ie: VSD, PDA, endocardial cushion defect
Etiology of heart failure in infants and toddler
- L to r shunt
- AVM - hemangioma
- anomalous left coronary artery from PA
- genetic or metabolic cardiomyopathy
- acute HTN
- SVT
- KD
- myocarditis
122
X-ray of cardiomegaly and boy has dyspnea, increased JVP, S3, S4, pansystolic murmur at the apex.
A. What are two findings on the X-ray ?
B. How do you interpret this X-ray ?
C. What is the most likely cause of this presentation ?
A. 2x finding on X-ray
1. Cardiomegaly
2. Increased pulmonary vascular markings
B. How do you interpret this X-ray ?
- congestive heart failure
C. What is the most likely dx ?
- VSD
This is congestive heart failure.
Pansystolic murmur = TR, MR, VSD
Large VSDs with excessive pulmonary blood flow = dyspnea, feeding difficulties, poor growth, duskiness with crying, diaphoresis, CHF in early infancy
CXR findings in large VSD
1. Cardiomegaly
2. Increased pulmonary vascular markings
3. Frank pulmonary edema or pleural effusions
123
Description of TGA
A. What is the diagnosis ?
B. What do you give next ?
Extra
C. What is surgery for TGA ?
D. Describe auscultory and CXR findings of TGA
A. TGA
B. Prostaglandins
C. Arterial switch procedure - jatene
D. Auscultory findings
- Intact septum- single s2, +/- soft SEM
- VSD - grade 3-4 holosystolic murmur, S3 gallop, diastolic rumble
CXR- mild cardiomegaly and narrow mediastinum - egg on a string. Normal to increased pulmonary blood flow
124
Child with syncope X2, has long QT, what 3 historical information would make you concerned ?
Long QT should be considered high risk when it is associated with the following:
1. Congenital deafness (Jervell- Lange- Nielsen syndrome)
2. Recurrent syncope due to malignant ventricular tachyarrhythmia
3. Family history of sudden death
4. QTc > 500ms
5. 2:1 atrioventricular block
6. T wave electric alternans
7. LQTS3 genotype
125
Name 2 cardiac conditions that have a single S2
1. Truncus arteriosus
2. HLHS
3. TOF
4. TGA
5. Pulmonary or aortic atresia or severe stenosis
126
Name the cardiac lesions that have
| Splitting of S2
1. ASD
2. Pulmonary stenosis
3. Epstein anomaly
4. TAPVR
5. RBBB
127
Work up of patient with vascular ring ?
Extra
Name the types of vascular rings
1. Cardiac MRI has now replaced barium esophagram
Cardiac catherization is reserved for cases with associated anomalies or in rare cases where these other modalities are not diagnostic
Vascular ring - vascular anomalies that result from abnormal dvlp of the aortic arch complex.
Double aortic arch is the mc complete vascular ring, encircling both the trachea and esophagus, compressing both
2. Types
1. Double arch - mc
2. Right arch and ligamentum/ductus
3. Anomalous innominate
4. Aberrant right subclavian
5. Pulmonary sling
128
Newborn with persistent bradycardia. ECG given. Looks like heart block
A. Identify the problem
B. What 2 things is this child at risk for ?
A. Complete AV block (autoimmune 70% vs congenital vs structural defect
B. What two things at risk for ?
1. Associates congenital heart disease
2. Heart failure
3. Cytopenias and rash with neonatal lupus
- Autoimmune disease accounts for 60-70% of all cases of congenital complete heart block and around 80% of cases in which the heart is structurally normal.
- Also seen in pts with complex congenital heart disease and abnormal embryonic development of the conduction system.
- Can be associated with ASDs -genetic type,
- myocardial tumors,
- myocarditis,
- myocardial abscess secondary to endocarditis
- genetic abnormalities including LQTS and Kearn- Sayre syndrome
- complication of congenital heart disease repair and in particular repairs involving VSD closure
129
An infant presents with a 2 day history of poor feeding, more fussy. In the ER, ECG is done (given- shows a narrow complex QRS, tachycardia, rate about 240, p waves not consistently seen).
A. What is the rate ?
B. What is your diagnosis ?
C. The child looks stable. What are two things you can do to treat ?
1. HR - 240
2. Diagnosis - SVT
3. Two things to treat in stable child ?
A. Vagal maneuver
B. Adenosine
If unstable then synchronized cardioversion
130
A teenage boy, previously healthy, presents with a 2-3 day history of SOB and diaphoresis. There is no fever. A CXR is done (showed cardiomegaly, increased perivascular markings, no pneumothorax or pneumomediastinum). Hepatomegaly is noted on exam.
A) what two things do you note on the CXR ?
B. What are the two most likely causes that you need to think of ?
```
A). CxR = cardiomegaly and increased perivascular markings
B). Two most likely causes
- congestive heart failure beyond infancy is unusual in patients with CHD and suggests a complicating factor
1. Dilated cardiomyopathy - Mx
2. Infected endocarditis
3. Myocarditis
4. Valvular regurgitation
5. Severe Anemia
6. Rheumatic fever
7. Tachyarrhythmia
```
131
Child with fixed split S2 and a systolic ejection murmur on LUSB.
A. What is diagnosis
B. List 2 other conditions that would give you a fixed S2
```
A. Dx is ASD
B. 2 other conditions give fixed S2
1. TAPVR
2. Epstein anomaly
3. Pulmonary stenosis
4. RBBB
5. Coronary sinus septal defect ?
6. Large AVMs ?
```
132
Patient presents with syncope with exercise. What would make you suspect the more common (Romano-ward syndrome) prolonged QT syndrome ?
Family history - as autosomal dominant and known cases of or hx of syncope, sudden death, cardiac arrest.
133
Name three cyanotic heart defects with low pulmonary vasculature
1. TOF
2. Pulmonary atresia or severe pulmonary stenosis
3. Tricuspid atresia
4. Epstein’s anomaly
5. DORV with pulmonary stenosis
134
Picture of a CXR with a large heart. A 16 year old patient presents with chest pain which is better when she leans forward. Her heart rate is 98. She has pulsus paradoxus of 15.
A. Describe the CXR findings
B. What is the likely diagnosis ?
A. Cardiomegaly
B. Pericarditis +/- tamponade
The most common symptom of acute pericarditis is chest pain, typically described as sharp/stabbing, positional, radiating, worse with inspiration, and relieved by sitting upright or prone. Cough, fever, dyspnea, abdominal pain, and vomiting are non-specific symptoms a/w pericarditis. Additionally signs and symptoms of organ involvement may occur in the presence of generalized systemic disease.
Muffled or distant heart sounds, tachycardia, narrow pulse pressure, jugular venous distensionand a pericardial friction rub provides clues to the diagnosis of acute pericarditis. Cardiac tamponade is recognized by the excessive fall of systolic BP > 10mmHg with inspiration.
Other conditions with may result in pulsus paradoxus include severe dyspnea, obesity, and PPV.
135
List 3 signs of SVT on the ECG of a 2 year old.
1. Tachycardia
2. Narrow QRS
3. Absent p waves
Life in the fast lane
- Tachycardia > 220 infant and > 180 child
- QRS complexes usually narrow <120ms unless pre- existing bundle branch block
- ST segment depression may be seen with or without underlying coronary artery disease
- QRS alternans - physic variation in QRS amplitude a/w AVNRT and AVRT distinguished from electrical alternans by a normal QRS amplitude.
- p waves if visible exhibit retrograde conduction with p- wave inversion in leads II, III, aVF.
- p waves may be buried in the QRS complex, visible after the QRS complex or very rarely visible before the QRS complex
136
Infant with cyanosis, sats 75%, no murmur, murmur but single s1 and S2. Chest sounds clear. CXR shows with sketchy “egg on a string “
TGA
137
List four clinical signs of endocarditis in a patient with a fever and a new murmur. How do you confirm the diagnosis ?
A. List 4 clinical signs of endocarditis
- Roth spots
- janeway lesions
- Splinter hemorrhages
- glomerulonephritis
- fever
- new heart murmur
- petechia
- HSM
- Osler nodes
- conjunctival hemorrhages
B. How do you confirm the diagnosis ?
Path criteria:
- micro- organisms demonstrated by culture or histological exam of vegetation, vegetation that has embolized or intracranial abscess
OR
- pathologic lesions: vegetation or intracranial abscess confirmed by histologic exam showing active IE
Clinical criteria
2 major criteria OR
1 major + 3 minor OR
5 minor
138
Cyanotic baby, presents at 5 days of age. His data increase from 79 to 81 with oxygen. His RR - 50s, HR 180s. On exam he has a single S2 loud, no murmur. CXR shows narrow mediastinum and mildly increased pulmonary markings. EKG shows mild RV hypertrophy.
A. What is your diagnosis ?
B. What is one medication you can give for treatment ?
C. What are 4 complications of this treatment ?
A. Cyanotic heart disease with increased pulmonary blood flow ie; TGA, Truncus arteriosus, single ventricle lesion or TAPVR
B. Treatment - PGE
C. 4 side effects of PGE
FHATs
```
Fever
Hypotension, hypokalemia
Apnea
Tachycardia
Seizure
```
139
First line treatment of a child with pulse less ventricular tachycardia
Defibrillate 2J unsynchronized
140
Teen at a concert and feels dizzy and then passes out briefly. You suspect that this is vasovagal syncope. List 4 signs and symptoms that would make you suspicious for a cardiac origin of syncope
1. Symptoms during the exercise
2. No prodrome
3. Chest pain or palpitations a/w syncope
4. Family history of sudden death
141
4 risk factors for artherosclerosis
1. Obesity
2. HTN
3. Hyperlipidemia
4. Smoking
142
Match the arrhythmia with the first line medication
1. Asystole
2. Bradycardia
3. SVT
4. Ventricular tachycardia with a pulse
5. Ventricular tachycardia without a pulse
6. Pulseless electrical activity
A. Epinephrine 1:10000; 0.1ml
B. Adenosine (0.1 mg/kg)
C. Lidocaine
D. Amiodarone
1. Asystole = epinephrine
2. Bradycardia = epinephrine
3. SVT = adenosine
4. V tach with pulse = adenosine or maybe amiodarone
5. V tach with no pulse = epinephrine
6. Pulseless electrical activity = epinephrine
143
Neonate with complete heart block. Echo normal. List 2 causes
1. Maternal SLE - neonatal lupus
2. Myocarditis
3. Genetic mutation of NKx2-5 - congenital AV block in A/w ASD
4. Complex congenital heard disease
5. Abnormal embryonic dvlp of conduction system
6. Myocardial tumors
7. Myocardial abscess secondary to endocarditis
8. LQTS
9. Kearn- Sayre syndrome = mitochondrial myopathy, ocular involvement
10. Complication of CHD repair especially VSD
