Cardiology

Question 1

Tetralogy of Fallot • What determines the physiology in tetralogy of Fallot?
A. The size of the ventricular septal defect.
B. The position of the ventricular septal defect.
C. The presence of an atrial septal defect.
D. The degree of RV outflow tract obstruction.
E. The presence of a left superior vena cava.

Answer 1

B. The position of the ventricular septal defect.

Question 2

Who needs endocarditis prophylaxis?

Answer 2

History of endocarditis constitutes one of the high risk groups
High risk groups include:
-Previous IE
-Prosthetic cardiac valve (20% risk of mortality if infected with viridans streptococcus)
-Unrepaired cyanotic CHD, including palliative shunts or conduits
-First 6 months following repair of CHD using prosthetic material (endothelialization usually occurs within the first 6 months)
-Repaired CHD with residual defects at the site or adjacent to the site of a prosthetic patch OR prosthetic device (which inhibit endothelialization)
-Cardiac transplant recipient with valvulopathy
-Dental procedures: high risk groups warrant prophylaxis for all procedures that involve manipulation of the gingival tissue, periapical region of the teeth or perforation of oral mucosa
Resp: incision/biopsy of respiratory mucosa e.g. tonsillectomy/adenoidectomy
-GI/GU: patients being treated for a GI/GU infection should have an antibiotic that covers for enterococcus (Amp or vancomycin), dont need for circ
-Skin: should have coverage against staphylococci and GAS. Penicillin or cephalosporin, if allergic use vancomycin or clindamycin. If MRSA suspected vancomycin is recommended.

Question 3

What is the most common presentation of a 2-day old newborn with cyanotic heart disease?

a. bounding/dynamic precordium
b. normal pulses and quiet precordium
c. decreased pulses and poor perfusion
d. tachypnea and nasal flaring
e. palpable thrill

Answer 3

b. normal pulses and quiet precordium

Question 4

PGE1 being started for a duct-dependent lesion in a newborn. Which of the following is the following is MOST important to monitor for?
Hypertension
Hypoglycemia
Hypoventilation
Lactic acidosis

Answer 4

hypoventliation

The initial dose is dependent on the clinical setting, as the risk of apnea, one of the major complications of prostaglandin E1 infusion, is dose dependent.
Initial dose of 0.01 mcg/kg/min if the duct is known to be large (typically used for infants who are known to have a large PDA by echo)

Question 5

A teenager has long QT syndrome. He also has been having syncopal episodes while participating in sport. What is the best course of management:

a. Start CCB and restrict vigorous activity
b. Start CCB and do not restrict activity
c. Start beta blocker and restrict vigorous activity
d. Start beta blocker and do not restrict activity

Answer 5

c. Start beta blocker and restrict vigorous activity

Question 6

5 year old with exercise intolerance. On examination he has a slight heave at the left lower sternal 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) MS
b) MVP
c) AS
d) PS

Answer 6

d- PS

a. Mitral stenosis - rumbling diastolic murmurs, 2nd heart sound is loud and split.
b. Mitral valve prolapse - late systolic apical murmur preceded by a click, can see biphasic T waves in leads II, III, AVF. Not progressive, no therapy recommended, antibiotic prophylaxis no longer recommended.
c. Aortic stenosis - murmur usually heard along RIGHT upper sternal border, children as usually asymptomatic but symptoms can include easy fatigability, exertional chest pain, syncope.
d. Pulmonary stenosis - systolic ejection murmur heard loudest in second left upper sternal border, radiates to the back and to the lung fields. Normal S1 followed by an audible pulmonic ejection click the closer the click to S1 the more severe the pulmonic stenosis. P2 becomes softer as the stenosis becomes more severe.

Question 7

A 5 year old girl is referred for assessment of a murmur heard by her family MD. She has a
coarse murmur heard in the right subclavicular area when sitting up, and disappears when
she lies down. What is the most likely diagnosis?
a. Stills murmur
b. Venous hum
c. Patent ductus arteriosus
d. ASD

Answer 7

B venous hum

PDA - continuous murmur, left upper sternal border.

Atrial septal defect — systolic murmur in left upper sternal border caused by increased flow across pulmonary valve (reason why it is during systole) secondary to RV volume overload due to to L–>R shunting . Occasionally, the only abnormality is a persistently fixed split S2.

The innocent Still’s murmur is a systolic murmur with maximum intensity at the left lower sternal border or between the left lower sternal border and apex, and has minimal radiation (movie 8). The murmur has a characteristic vibratory or musical quality, is louder in the supine than sitting position, is louder in hyperdynamic states (fever, anxiety), and usually is grade 1 or grade 2 in intensity. This murmur usually resolves by early adolescence

Question 8

When would endocarditis prophylaxis be required for a patient with a history of endocarditis?

a. Dental cleaning
b. Umbilical piercing - no, only for procedures on infected skin should cover for GAS and staph.
c. Appendectomy - no prophylaxis no longer recommended for GI/GU procedures.
d. Myringotomy + tubes - This applies to individuals who undergo an invasive procedure of the respiratory tract that involves incision or biopsy of the respiratory mucosa, such as tonsillectomy and adenoidectomy.

Answer 8

a. Dental cleaning

History of endocarditis constitutes one of the high risk groups
High risk groups include:
Previous IE
Prosthetic cardiac valve (20% risk of mortality if infected with viridans streptococcus)
Unrepaired cyanotic CHD, including palliative shunts or conduits
First 6 months following repair of CHD using prosthetic material (endothelialization usually occurs within the first 6 months)
Repaired CHD with residual defects at the site or adjacent to the site of a prosthetic patch OR prosthetic device (which inhibit endothelialization)
Cardiac transplant recipient with valvulopathy
Dental procedures: high risk groups warrant prophylaxis for all procedures that involve manipulation of the gingival tissue, periapical region of the teeth or perforation of oral mucosa
Resp: incision/biopsy of respiratory mucosa e.g. tonsillectomy/adenoidectomy
GI/GU: patients being treated for a GI/GU infection should have an antibiotic that covers for enterococcus (Amp or vancomycin)
Skin: should have coverage against staphylococci and GAS. Penicillin or cephalosporin, if allergic use vancomycin or clindamycin. If MRSA suspected vancomycin is recommended.

Question 9

A 30 month hold is found to have a normal S1 and fixed, split S2 on auscultation with a 2/6
murmur at the left upper sternal border. What is his ECG likely to show?
a. Prolonged PR interval
b. Signs of RV overload
c. Left bundle branch block

Answer 9

B signs of RV overload

Widely split S2 is caused by conditions that delay closure of the pulmonic valve. E.g. pulmonary stenosis.
A fixed split S2 is caused by conditions that increase RV volume, the most common cause is an ASD.

Question 10

Newborn baby has a murmur. What do you do?

a. Follow closely
b. Send to family MD
c. Urgent cardio consult

Answer 10

a) follow closely

Question 11

How do you treat a child with hypertrophic cardiomyopathy?

a. Beta blocker
b. ACE inhibitor
c. Furosemide

Answer 11

BETA blocker

beta blockers or calcium channel blocking agents (verapimil) may be useful in diminishing ventricular outflow tract obstruction, modifying ventricular hypertrophy, improving ventricular filling.
Beta blockers or calcium channel blockers do not reduce the risk of sudden death OR the risk of developing heart failure.

Question 12

153. A 14y old is being medically evaluated for high school 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

Answer 12

a - ECG (good screen for both LQTS and HOCM)

Question 13

A 6y old child is vomiting and has abdominal pain 3w ago she had repair of a secondum ASD. Findings include listlessness and pallor. Temp 40, HR140, BP85/52 RR 36/min, Mild tenderness to deep palpation of the abdomen and decreased intensity of femoral pulse as she inhales. The most appropriate initial study:

a. Abdominal US
b. cbc and blood culture
c. Echo - septic emboli - actually not LOL
d. ECG

Answer 13

C- echo
Septic emboli vs-
Post pericardiotomy syndrome - Immune phenomenon that occurs days to months (usually 1–6 weeks[1]) after surgical incision of the pericardium (membranes encapsulating the human heart)

Question 14

Tetralogy of Fallot • What determines the physiology in tetralogy of Fallot?
A. The size of the ventricular septal defect.
B. The position of the ventricular septal defect.
C. The presence of an atrial septal defect.
D. The degree of RV outflow tract obstruction.
E. The presence of a left superior vena cava.

Answer 14

D. The degree of RV outflow tract obstruction.

Question 15

A newborn has cyanosis. His CXR is as follows: (CXR shows slightly boot shaped heart with narrow mediastinum, oligemic lungs)

a. TGA
b. TAPVR
c. Truncus arteriosus
d. TOF

Answer 15

TOF

Question 16

11 yo male who is obese. His father had a myocardial infarction at the age of 38 years. His total cholesterol is 6.3 and his LDL is 3.8. What is the best management?

a. lifestyle modification
b. lifestyle modification and low-fat diet
c. lifestyle modification and bile acid sequestrant
d. lifestyle modification and statin

Answer 16

b. lifestyle modification and low-fat diet

Trial for 6 months
Can tx if >10yo after that

Question 17

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

Answer 17

a) asymptomatic

Even an extremely large secundum ASD rarely produces clinically evident heart failure in children. Subtle FTT in younger children, older children may have varying degrees of exercise intolerance.

Question 18

Young girl with AOM and fever for 9 days, now with conjunctivitis, cracked lips, etc…kawasaki. Which is the most important to test before she leaves?
a) Cardiac ultrasound

Answer 18

a) Cardiac ultrasound

HOT CREAM

Question 19

Murmur, LLSB, variable split S2, II/VI murmur

a) PS –
b) Bicuspid AV
c) Benign –

Answer 19

Still’s murmur’s can present in the LLSB, a variable split S2 is physiologic

PS left upper sternal border, the S2 would be split

Question 20

Heart lesion with liver, and cardiomegaly, and distress

a) Furosemide
b) Propranolol

Answer 20

A Furosemide

Question 21

Kid with elevated BP confirmed by ambulatory monitoring. what’s next?

a) Renal US
b) Treat with nifedipine
c) Recheck in a month
d) Ambulatory monitoring

Answer 21

Renal us’

If the BP is at the 95th percentile or greater, BP should be staged. Stage 1 is defined by BP between the 95th and 99th percentiles plus 5 mm Hg. Stage 2 is defined by BP > the 99th percentile plus 5 mm Hg (Grade D).
If BP is stage 1, BP measurements should be repeated on 2 more occasions within 1 month; if hypertension is confirmed, evaluation (as described in section IV. Routine Laboratory Tests for the Investigation of Children With Hypertension) or appropriate referral should be initiated within 1 month, or both (Grade D).
o ii. If BP is stage 2, prompt referral should be made for evaluation and therapy

Question 22

Unstable SVT, what to do

a) Amiodarone
b) Adenosine
c) Asynchronous Defib
d) Valsalva

Answer 22

adenosine

Best answer: synchronized cardioversion

Question 23

Child getting large volumes of PRBC transfusion. What ECG complication do you expect to see?
A-Peaked T-waves
B-U waves
C- Short PR interval

Answer 23

peaked T

Underlying cause: hyperkalemia
ECG findings associated with hyperkalemia [UTD]
§ Peaked T waves
§ Prolonged PR and QRS intervals and small P waves
§ Loss of P wave, further prolongation of QRS interval (“sine wave” pattern), and conduction delay that can manifest as bundle branch or atrioventricular (AV) nodal block
§ Ventricular fibrillation or asystole

Question 24

Which ECG change is characteristic of acute rheumatic fever?
a- Peaked T waves
b- Prolonged PR interval
c- Sinus tachycardia

Answer 24

Prolonged PR interval
Jones criteria

MAJOR
J jts
O looks lke a heart (carditis)
N-nodules subQ
E- erythema marginatum
S-sydenham corhea

MINOR
CAFEPAL
crp elevated
arthalria
fever
esr high
proloned PR
anamesis of rheumination
leukocytosis

NEED trhoat cultures with GABS or elevated anti strep O titires plus 2 MAJOR or 1 MAJOR and 2 minor

Question 25

Kid with soft murmur over left upper sternal border, and fixed split S2. Diagnosis?
a- Pulmonary stenosis
b= Mitral regurg
c- ASD

Answer 25

C ASD

Pulmonary stenosis: wide S2, heard over left upper sternal border, worst the stenosis, the quieter the second S2 is
ASD: most common cause of wide fixed split S2

Question 26

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.
Holter
Echocardiogram
ECG
??

Answer 26

ECHO for HCM

vaslava makes HCM worst for the mrumur

Question 27

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 of 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

Answer 27

A prolonged qt

Clinical manifestation of Long QT syndrome is usually a syncopal episode brought on by exercise, fright, sudden startle, some events occur during sleep

calculate QT/ sqrt RR (before theq t_

Question 28

bese 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. 24 hour Holter
B. ECG
C. Exercise test

Answer 28

A 24 hour holter

Holter monitor is the preferred ambulatory ECG monitoring test for patients with daily or near daily symptoms, and for patients in whom a comprehensive assessment of all cardiac activity over a 24 to 48 hour interval is required. Clinical scenarios in which a Holter monitor is a good choice include:
●Patients with syncope, near syncope, or dizziness occurring on a daily or near daily basis. ●Patients with palpitations occurring on a daily or near daily basis.
●Patients with atrial fibrillation (AF) for assessment of ventricular rate control.
●Patients with known AF (or another tachyarrhythmia) or frequent ectopy (either ventricular or atrial premature beats [VPBs or APBs]) associated with a reduction in left ventricular ejection fraction.
●Patients with acute coronary syndrome

Question 29

83) An 8 year old female presents after experiencing a syncopal episode while playing soccer. She had a 2 month old sibling who died from sudden infant death syndrome. Which ECG parameter would most likely be abnormal?
1) PR interval
2) QRS
3) QTc
4) ST segment

Answer 29

qtc

Question 30

Cyanotic infant. CXR shows large heart and oligemic lung fields (question describes a CXR, but no actual CXR attached). Which lesion?
ToF
Truncus arteriosis
TAPVD
ASD

Answer 30

TOF
VSD, PS/PA, overriding aorta, RVH

Other Cyanotic lesions with decreased pulmonary flow:
TOF (with pulmonary atresia, with pulmonary atresia with major aortopulmonary collaterals)
Pulmonary stenosis
Tricuspid atresia
Double outlet right ventricle

Question 31

4 day old male presents with acidosis (pH 7.10, PC02 28) with hepatomegaly and poor feeding. His oxygen saturations are 88% in room air. What is the next step in your management.
a Antibiotics
b Prostaglandins
c NPO and IV Fluids (D12.5)

Answer 31

prostoglandin

duct dependent lesion is suspected, an infusion of prostaglandin E 1 should be initiated immediately to maintain patency of the ductus arteriosus and improve oxygenation (dosage: 0.01-0.20 µg/kg/min)

Question 32

145. 18m old boy noted to have soft high pitch murmur Grade 2 on left sternal border with fixed split S2
What is the Diagnosis?
A. ASD
B. Truncus
C. ?
D. ?

Answer 32

ASD

Question 33

Teenage girl with recurrent syncope after prolonged standing. Has prodromal symptoms (lightheadedness, etc). What is the MOST likely diagnosis?
Neurocardiogenic
Long QT
Postural orthostatic tachycardia syndrome

Answer 33

neurocardiogenic

Question 34

151. (Repeat question): 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 palpable peripheral pulses. What is most appropriate next step?
Carotid massage
Adenosine
Asynchronous counter shock
Digoxin

Answer 34

adenosine

Question 35

96. 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?

Benign
Bicuspid aortic valve
VSD
Pulmonary stenosis

Answer 35

BENIGN

Still’s murmur: SEM, loudest at LLSB/apex, minimal radiation, vibatory/musical quality, louder supine than sitting and in hyperdynamic states (fever, anxiety), ages 3-7 (resolves by adolescence)
Venous hum: produced by turbulence of blood in jugular venous system, heard in neck or anterior portion of upper part of chest, humming sound in systole/diastole, decreases w/ compression of jugular venous system

Question 36

46. Teen girl with hypertension and BMI 30. What is best way to determine long term damage from the HTN?
a ECG
b Echo
c Serum creatinine
d Fundoscopy

Answer 36

B echo

Question 37

What is the most common presentation of a 2-day old newborn with cyanotic heart disease?

a. bounding/dynamic precordium
b. normal pulses and quiet precordium
c. decreased pulses and poor perfusion
d. tachypnea and nasal flaring
e. palpable thrill

Answer 37

B normal

Question 38

151. (Repeat question): 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 palpable peripheral pulses. What is most appropriate next step?
a Carotid massage
b Adenosine
c Asynchronous counter shock
d Digoxin

Answer 38

B adenosine

Question 39

96.  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

Answer 39

BENIGN

still murmur

Question 40

2. Child with hypertrophic cardiomyopathy, what do you treat with?
   a) Beta blocker
   b) Ace inhibitor
   c) Digoxin
   d) FurosemidePulmonary stenosis

Answer 40

beta blocker

Question 41

Infant presents unwell. HR 136, RR50. EKG shows Qwaves 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

Answer 41

Anomalous left coronary artery from the pulmonary artery

In anomalous origin of the left coronary artery from the pulmonary artery, the blood supply to the left ventricular myocardium is severely compromised. Soon after birth, as pulmonary arterial pressure falls, perfusion pressure to the left coronary artery becomes inadequate; myocardial ischemia, infarction, and fibrosis result.

electrocardiogram resembles the pattern described in lateral wall myocardial infarction in adults. A QR pattern followed by inverted T waves is seen in leads I and aVL. The left ventricular surface leads (V 5 and V 6 ) may also show deep Q waves and exhibit elevated ST segments and inverted T waves

Question 42

28. Which cardiac defect is most likely in a child with 22q11?
    a) TOF
    b) VSD
    c) Aortic coarctation

Answer 42

TOF

CATCH 22 = Cardiac defects (aortic arch, conotruncal), Abnormal facies, Thymic aplasia, Cleft palate, Hypocalcemia, 22q11 deletion

Question 43

57. Kid w fontan

a. Protein losing enteropathy

Answer 43

UTD: Protein losing enteropathy - severe protein loss through gut, hypoalbuminemia
Causes - hypertrophic gastritis (Ménétrier’s disease), milk protein allergy, celiac disease, inflammatory bowel disease, giardiasis, intestinal lymphangiectasia, and right sided heart dysfunction (post-Fontan procedure)
Screening test - stool for alpha-1 antitrypsin

Post-Fontan:
Secondary intestinal lymphangectasia - dilated intestinal lymphatics and increased lymph pressure

Question 44

What`s the most common cardiac lesion in a IDM?

a. HCM
b. ASD
c. VSD

Answer 44

HCM

Question 45

134. Infraclavicular murmur that is louder on sitting up

Answer 45

a. Venous hum

A cervical venous hum is also a very common innocent murmur in children. It is a continuous murmur audible at the left or right upper sternal borders, infraclavicular or supraclavicular regions. It is louder when the patient is sitting with head extended, and becomes softer or absent with rotation or flexion of the head or applying light pressure over the jugular vein when the patient is sitting, or when the patient is supine. The alteration of the murmur’s intensity with these maneuvers aids in distinguishing the murmur from a PDA.

Question 46

20. ECG tracing showing bradycardia/heart block. What are you most likely to find on maternal history?
    a. Parvo
    b. SLE

Answer 46

SLE

Question 47

A newborn is found to have a rash and anemia. An ECG is shown with what looks like complete heart block (dissociation between p waves and QRS; QRS rate = 6 blocks apart: HR 50). What is the mostly like illness that would be found in the mother?

a. SLE
b. Hyperthyroidism
c. Addison’s

Answer 47

SLE

Question 48

64. Girl with symptoms with high BMI and hypertension. Which of the following tests is MOST likely to reveal an underlying sequelae of her disease?

a. Echo
b. Ecg

Answer 48

ECHO

UTD: Left ventricular hypertrophy (LVH) is the most prominent manifestation of end-organ damage from HTN. LVH is associated with adverse cardiovascular disease (CVD) outcomes, and a significant number of children and adolescents with HTN have LVH [7-12]. Echocardiography is the recommended modality to detect LVH due to pediatric HTN. The 2017 American Association of Pediatrics (AAP) high blood pressure (BP) guidelines recommends echocardiography to assess for target-organ cardiac damage be performed at the time when pharmacologic therapy is being considered

Question 49

79. Boy with TAPVD. Want to start ADHD med. What do you do?
    a. Hx and PE and consult with cardiologist
    b. ECG
    c. Cardiology consult
    d. ?Echo

Answer 49

a = hx and pe and call cardio

CHD patients frequently have ADHD and can potentially benefit from ADHD therapies, including appropriately prescribed medication
Patients with ADHD, like all paediatric patients, should undergo a careful history and physical examination that includes personal and family history details that may identify those at risk of sudden cardiac death. This should be performed by their primary care physician
Routine ECG assessment of ADHD patients before starting medication is not supported by evidence and is not recommended
For ADHD patients 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.

Question 50

80. 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

Answer 50

b. Infections were more likely from exposures from everyday life and not that much more from dental procedures

Question 51

94. Soft second heart sound with click. Left parasternal heave. Systolic murmur left upper sternal border.
    a. pulmonary stenosis

Answer 51

CLICK = valvular issue
UTD: Pulmonic stenosis
The first heart sound is normal. In patients with mild or moderate PS, it is typically followed by an audible click. The closer the click to the first heart sound, the more severe the PS, until it merges with the first heart sound. he click corresponds to the time when the doming pulmonary valve reaches its open position
The split between the second heart sounds is dependent on the severity of the obstruction. The more the severity increases, the longer it takes the RV to empty and the wider is the splitting. The second component (pulmonary) is also decreased, and proportionally lower as the pressure in the pulmonary artery decreases in more severe PS.
The characteristic murmur of valvular PS is a systolic ejection murmur heard at the second left intercostal space. In general, the intensity of the murmur increases with the severity of the obstruction.

Question 52

37. 6 wk 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. propanolol
    b. furosemide
    c. digoxin
    D.adenosine

Answer 52

furosemide

Diuretics are usually the first mode of therapy initiated in pediatric patients with heart failure. Furosemide is the most commonly used. These agents reduce the circulating blood flow and thereby reduce pulmonary fluid overload and ventricular filling pressure.
Other diuretics used are Spironolactone (potassium sparing, eliminates need for K supplements as with Furosemide) and Chlorothiazide
ACE inhibitors or ARBs (afterload reducers) are useful in patients with heart failure secondary to severe cardiomyopathy or with severe mitral/aortic insufficiency; they are usually added in addition to diuretics
Digoxin, once a mainstay in heart failure of both children and adults, is now used much less frequently due to new agents and recognition of potential toxicities
Beta blockers are used for chronic treatment of patients with heart failure and should not be administered when patients are in the acute phase of heart failure

Question 53

3 week? old baby with tachypnea RR 70, chest crackles, diaphoresis with feeding. Grade III/VI murmur pansystolic, liver at 5 cm below costal margin. Treatment:

a. digoxin
b. furosemide
c. prostaglandin

Answer 53

furosemide

Question 54

130. Baby is refusing to feed since this morning. He is pale and lethargic over the last 24 hrs. HR 300, RR 70, weak pulses. What do you do? Baby weight 8kg
     a. synchronized 4J cardioversion (if starting on lower range)
     b. synchronized 8J cardioversion (but could technically also be correct)
     c. asynchronized 4J cardioversion
     d. asynchronized 8J cardioversion

Answer 54

a. synchronized 4J cardioversion (if starting on lower range)

PALS Tachycardia Algorithm
We don’t know if narrow complex or wide complex tachycardia
If narrow complex (<0.09 sec), most likely SVT (given rate >220 bpm) therefore if IV access can give adenosine, or if no IV access/adenosine ineffective then give synchronized cardioversion
If wide complex (>0.09 sec) and signs of cardiopulmonary compromise (hypotension, altered mental status, signs of shock), then give synchronized cardioversion
Dose: begin with 0.5 - 1.0 J/kg. If not effective, increase to 2 J/kg

Question 55

45. 5 day old with tachycardia, tachypnea, cyanosis and left SaO2 is 91% and rights SaO2 is 98%. Best next step is:
    a. start PGE1
    b. give indomethacin
    c. intubate
    d. chest xray

Answer 55

PGE

Question 56

Newborn diagnosed with interrupted aortic arch, what to start?

a. dopamine
b. prostaglandin
c. nitric oxide
d. Indomethacin

Answer 56

PGE

interupted aortic arch - duct dependant
think about digeorge

Question 57

123. In the most common long QT syndrome (Romano-Ward), you ask about:
     a. Deafness
     b. Family history

Answer 57

B family hx

●The more common autosomal dominant form, the Romano-Ward syndrome, has a purely cardiac phenotype
●The autosomal recessive form, the Jervell and Lange-Nielsen syndrome, is associated with LQTS and sensorineural deafness, and a more malignant clinical course

Question 58

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

Answer 58

d fmhx

because of AD inheritance pattern

Question 59

16. Long QT associated with?
    a. hypokalemia
    b. hypercalcemia
    c. clarithromycin
    d. digoxin

Answer 59

a/c

Nelson’s: Electrolyte disturbances that prolong QT interval
Hyopokalemia
Hypocalcemia
Hypomagnesemia

Question 60

30. Infant with R arm sat of 90% and L leg sat of 70%. Pt tachypneic, RR 70, no distress. Dx?
    a. CoA
    b. Truncus
    c. TGA
    d. TOF

Answer 60

a) CoA

Truncus: no differential
TGA: if VSD present, mixing occurs at that level therefore no differential. If intact ventricular septum present with severe cyanosis. If TGA with PHTN, coarctation or interrupted aortic arch, can see reverse differential cyanosis (higher spO2 post ductal)
TOF: no differential

UTD: Differential cyanosis — In infants with critical COA, interrupted arch, or critical aortic stenosis, differential cyanosis occurs, wherein the flow of deoxygenated blood through the ductus arteriosus supplies the lower half of the body’s circulation, but oxygenated blood flow from the left heart supplies the upper body via the vessels proximal to the site of arch obstruction

Question 61

24 hour infant with preductal sats 90%, post ductal sats 70% and RR 70/min

a. coarctation of aorta
b. truncus
c. TGA
d. TOF

Answer 61

a. coarctation of aorta

Preductal coarctation: The narrowing is proximal to the ductus arteriosus. Blood flow to the aorta that is distal to the narrowing is dependent on the ductus arteriosus; therefore severe coarctation can be life-threatening. Preductal coarctation results when an intracardiac anomaly during fetal life decreases blood flow through the left side of the heart, leading to hypoplastic development of the aorta. This is the type seen in approximately 5% of infants with Turner syndrome.[4][5]
Ductal coarctation: The narrowing occurs at the insertion of the ductus arteriosus. This kind usually appears when the ductus arteriosus closes.
Postductal coarctation: The narrowing is distal to the insertion of the ductus arteriosus. Even with an open ductus arteriosus, blood flow to the lower body can be impaired. This type is most common in adults. It is associated with notching of the ribs (because of collateral circulation), hypertension in the upper extremities, and weak pulses in the lower extremities. Postductal coarctation is most likely the result of the extension of a muscular artery (ductus arteriosus) into an elastic artery (aorta) during fetal life, where the contraction and fibrosis of the ductus arteriosus upon birth subsequently narrows the aortic lumen.[6]

Question 62

47. For which patient is prophylactic antibiotics before a dental procedure indicated for?
    a. Unrepaired TOF
    b. VSD that was repaired 10 months ago
    c. VSD that was repaired 8 months ago with a small residual leak
    d. Moderate aortic stenosis

Answer 62

unrepaired TOF

VSD repaired 8 mo ago with residual leak

Question 63

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 ventricle ejection

Answer 63

d. prolonged right ventricle ejection

Question 64

Tall adolescent male with height at the 95%, weight at he 75% and arm span greater than length. What is the most likely finding on echocardiogram?

a. Bicuspid aortic valve
b. Dilatation of the ascending aorta

Answer 64

b- dilation of AA

also get MVP
and root dilation

Marfan Syndrome
UTD: Dilatation of the aorta is found in approximately 50 percent of young children with MFS and progresses with time. Approximately 60 to 80 percent of adult patients with MFS have dilatation of the aortic root

Question 65

6y old boy, for check up and find on auscultation single s1 and wide, fixed split s2 with systolic mumur loudest at the LUSB. He appears well and is in no distress. Most likely explanation for the findings:

a. ASD
b. pulmonic stenosis
c. TAPVR

Answer 65

ASD

ASD: wide, fixed S2 split
Pulmonary stenosis: normal S1, normal split S2 (may be wide), pulmonary ejection click.
TAPVR: gallop rhythm (not usually a murmur)
AS: Loud harsh SEM at the RUSB radiating to the neck/left sternal border, diminished S1, systolic ejection click

Question 66

80. 12h old Newborn has a sat of 80%. Increases to 85% with 100% 02. Mild tachypnea rr65. CXR has no abnormalities. What next initial management should be done?
    a. Intubate and ventilate
    b. Prostaglandins
    c. Antibiotics

Answer 66

PG

Question 67

93. 15 y o boy for regular check up and exam shows Ht > 95th, Wt 50%, arm span > ht, + pectus, flat feet. What is the most likely cardiac defect that could be found.
    a. Mitral valve prolapse
    b. Bicuspid Ao valve
    c. Dilatation ascending aorta
    d. VSD

Answer 67

a vs C (root vs ascending aorta0

Question 68

12 hour old newborn with cyanosis, sats 80% increased to 85% on 100% O2. CXR normal pulmonary vasculature and no other anomalies. Most likely dx?

a. HLHS
b. TOF (would not have a normal CXR)
c. TGA
d. Tricuspid Atresia

Answer 68

TGA

Question 69

A newborn baby presents with tachypnea and O2 sats of 80%. Sats increase to 85% on 100% O2. CXR reveals normal pulmonary vasculature and was otherwise normal. Most likely diagnosis:

a. HLHS
b. tricuspid atresia
c. transposition of the great vessels
d. Tetralogy of Fallot

Answer 69

TGA

increased pulmonary vascularity
total anomalous pulmonary venous return (TAPVR) (types I and II)
transposition of the great arteries (TGA)
truncus arteriosus (types I, II and III)
large AVSD
single ventricle without pulmonary stenosis
decreased pulmonary vascularity
tetralogy of Fallot
pentalogy of Cantrell

Question 70

32. Infant of 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

Answer 70

resolve

Question 71

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-blocker

Answer 71

spntenous resolution

Question 72

At a regular office visit, a 7 y.o. girl is noted to have a continuous murmur in the right subclavicular area (or supraclav?), that is louder when upright. She is well and has no other contributory medical history.

a. Still’s murmur
b. PDA
c. Venous hum

Answer 72

venous hum

Question 73

122. 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

Answer 73

svt

Question 74

136. 5 year old kid comes in with pneumonia. You discover a loud S1, fixed S2 & a murmur at the LUSB. Most likely diagnosis?
     a. ASD
     b. PDA
     c. PS

Answer 74

AsD

Question 75

Child on risperdol for Tourette syndrome has frequent syncopal episodes with exertion. What is the cause?

a. hypoglycemia
b. Prolonged QT

Answer 75

risperidone: Bradycardia, bundle branch block, buttock pain, chest pain, ECG changes, facial edema, first degree atrioventricular block, hypertension, hypotension, orthostatic hypotension, palpitations, paresthesia, peripheral edema, prolonged Q-T interval on ECG, syncope, tachycardia.

Question 76

24. Infant with 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

Answer 76

. pulmonary arterial pressures have increased

Question 77

4. Well new born 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 for now

Answer 77

nothing for now

Question 78

Endocarditis prophylaxis would be needed in

a. Small ASD
b. Small VSD
c. Kawasaki
d. Mitral valve prolapsed

Answer 78

none from new guidelines

Question 79

29. A 3 day old is tachypneic, cyanosed despite 100% O2. Bilateral crackles on exam with weak peripheral pulses and no heart murmur. What is the diagnosis:
    a. HLHS
    b. Sepsis
    c. AV fistula

Answer 79

HLHS

Question 80

91. 2 month old baby with poor feeding, hepatomegaly, crackles, soft systolic murmur. What is the most likely diagnosis?
    a. congenital infection
    b. CHF
    c. Sepsis

Answer 80

CHF

Question 81

94. 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. late eruption of the right atrium
    e. prolonged right ventricle ejection’

Answer 81

E prolonged RV ejection

Question 82

ou 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. echocardiogram
d. ECG
e. electrolytes

Answer 82

ecg

Question 83

152. A 10 y underwent valvotomy for aortic stenosis 7y ago. He developed varicella 11days ago and now presents with a 6 day history of lethargy, anorexia, wt 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

Answer 83

B - IE

Question 84

8. Cardiac prophylaxis would be indicated in which of the following:
   a. Coarctation of aorta repaired 6 months ago
   b. asymptomatic ostium secundum
   c. asymptomatic and small VSD
   d. Kawasaki aneurysm
   e. lupus carditis

Answer 84

Coarctation of aorta repaired 6 months ago (if there was a conduit/stent)

Question 85

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 a congenital heart lesion
d. diagnosis is confirmed by ECG -
e. may be present in infants born to mothers with SLE -

Answer 85

if you hear a systolic murmur then it is associated with a congenital heart lesion

Question 86

44. 3 day infant cyanosis with crying, investigation (if next step ABG, for diagnosis ECHO)
    a. ECG
    b. CXR
    c. ABG - if this is hyperoxia test i would pick this
    d. bld cx
    e. echo

Answer 86

hyperoxia test

Question 87

40. An infant is in shock with paroxysmal supraventricular tachycardia. You would give:
    a. bag of ice to face
    b. synchronous DC cardioversion
    c. asynchronous cardioversion
    d. verapamil
    e. digoxin

Answer 87

B synchronous cardiovesio

Question 88

65. 3-day-old infant with congenital heart disease whose cyanosis is aggravated by crying. Most likely:
    a. TGA
    b. VSD
    c. ASD
    d. PDA
    e. PS

Answer 88

PS

like a TOF

Question 89

. 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

Answer 89

E PG

Question 90

SVT in neonates is most commonly associated with:

a. hypocalcemia
b. hyperthyroidism
c. electrolyte disturbances
d. ventricular septal defect
e. structurally normal heart

Answer 90

e - normal

Question 91

An ECG is shown. Left axis deviation, increased forces, ST-T changes with T-wave inversion.

a. LVH
b. RVH
c. heart block
d. Wolff-Parkinson-White
e. ST-T changes associated with digoxin therapy

Answer 91

E- digox

Question 92

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

Answer 92

B endocarditis

Janeway lesions = macular, nonpainful, erythematous lesions on the palms and soles
Osler’s nodes = painful violaceous nodules found in the pulp of fingers and toes
—–
Duke Criteria for diagnosis of endocarditis: 2 major, 1 major + 3 minor, or 5 minor
MAJOR:
1. positive blood cultures (at least 2 separate cultures)
2. evidence of endocarditis on echo
MINOR:
1. predisposing conditions
2. fever
3. embolic-vascular signs
4. immune complex phenomena (glomerulonephritis, arthritis, rheumatoid factor, Osler nodes, Roth spots)
5. single positive blood culture or serologic evidence of infection
6. echo signs not meeting major criteria

Question 93

You are seeing a teenager with a history of recurrent syncopal episodes. What is the best screening test for prolonged QT syndrome.

a. EKG
b. exercise EKG
c. Holter monitor
d. echocardiogram
e. electrolytes

Answer 93

EKG

Highly indicative: HR-corrected QT interval = >0.47 sec
Suggestive: HR-corrected QT interval = >0.44 sec
Associated with malignant ventricular arrhythmias - torsades de pointes and ventricular fibrillation. Treat with beta-blocker. Some require pacemaker.

Question 94

An infant is referred for evaluation of a heart murmur heard on day 2 of life. Birth history is unremarkable. Feeding well. Good colour and perfusion with RR 50. Grade II/VI systolic murmur heard at the left sternal border. Your plan:

a. send back to family doctor with no follow-up (
b. follow closely
c. cardiology consult
d. urgent echocardiogram
e. start prostaglandin infusion

Answer 94

CLSE F/u

(danielle grp - could be PDA and if persists = bad)

PDA vs per pulm stenosis, otherwsie well)

Question 95

A full term infant is diagnosed with meconium aspiration syndrome. He is desaturating despite 100% oxygen. An echocardiogram shows normal cardiac anatomy and R* L shunting at the ductus and atrial level. Which of the following interventions may be effective:

a. dopamine
b. indomethacin
c. nitric oxide
d. prostaglandins

Answer 95

NO

Question 96

Which of the following pulse profiles matches the diagnosis given:

a. pulsus alternans-constrictive pericarditis (usu pulses paradoxis)
b. atrial fibrillation-hypothyroidism (usu hyperthroid)
c. pulsus bigeminus-digoxin toxicity
d. dicrotic pulse-mitral stenosis

Answer 96

C

pulsus bigeminus-digoxin toxicity

Question 97

131. What is most characteristic of a Still’s murmur:
     a. vibratory murmur
     b. increases with sitting
     c. often radiates to axilla

Answer 97

vibratory

2-6yo, not usually infants -innocent systolic murmur with maximal intensity at the left lower sternal border or between the LLSB and apex, has minimal radiation. Characteristic vibratory or musical quality, louder supine than sitting, louder in hyperdynamic states (fever, anxiety), and usually grade 1-2 intensity. Usually resolves by early adolescence

Question 98

108. Picture of narrow complex tachycardia, no palpable pulse with poor perfusion:
     a. Carotid massage
     b. Asynchronous cardioversion (synchronous?)
     c. Adenosine
     d. Verapamil

Answer 98

adnosine

Question 99

What is stage 1 vs 2 HTN

what is end organ w./u

Answer 99

HTN defined
Stage 1 >95th percentile+5mmhg, stage 2 >99th+5mmHg
if stage 1- can rpt 2 more times over 1 month,. stage 2 tx right away

w.u- lytes, cr urea, Renal US, urine dip

CVS risk, fasting BG, Serum total cholesterol and high-density lipoprotein cholesterol, low-density lipoprotein cholesterol, and triglyceride levels (Grade C).

routine end organ, echo, ACR ratioin AM , retinal exam

Question 100

what is the management of HTN

What is therapeutic options

Answer 100

Pharmacologic therapy should be initiated when patients have:
• Symptomatic hypertension (Grade D);
• Hypertensive target organ damage (Grade C);
• Stage 2 hypertension (Grade D);
• BP is at or greater than the 90th percentile associated with diabetes mellitus type 1 or 2, chronic kidney disease, or heart failure (Grade D);
• Stage 1 hypertension without target organ damage that persists (≥ 6 months) despite a trial of nonpharmacologic therapy (Grade D).
Guidelines
• 1.Initial therapy should be monotherapy
An angiotensin-converting enzyme (ACE) inhibitor
An angiotensin receptor blocker (ARB) (Grade C); or
A long-acting dihydropyridine calcium channel blocker
An alternate option is a β-blocker (Grade D) although they are less preferable because of the side effect profile in children.

If BP goals are not achieved with standard-dose monotherapy for ≥ 6 months, children should be referred to an expert in pediatric hypertension (Grade D

ACE inhibitors (Grade C) and ARBs (Grade D) are not recommended as first-line agents in black patients and β-blockers are not recommended as first-line agents in children with asthma, diabetes (type 1 or type 2), or in high-performance athletes (Grade D).