CARDIOLOGY

Question 1

Heart defect in DiGeorge

Answer 1

Aortic arch defects

Question 2

Heart defect in Down Syndrome

Answer 2

AV canal defect

Question 3

Heart defect in Marfan syndrome

Answer 3

Aortic root dissection / Mitral valve prolapse

Question 4

Heart defect in William syndrome

Answer 4

Supravalvular aortic stenosis

Question 5

Heart defect in Noonan syndrome

Answer 5

Supravalvular pulmonic stenosis

Question 6

Heart defect in Turner syndrome

Answer 6

Coarctation of the aorta

Question 7

Heart defects in Fetal alcohol syndrome (FAS)

Answer 7

Septal defects (VSD, ASD etc.)

Question 8

Treatment for dilated cardiomyopathy with heart failure

Answer 8

Diuretics + ACEi

Question 9

What is pulsus paradoxus? Which condition is it associated with?

Answer 9

Pulsus paradoxus = DECREASE in systolic arterial pressure during INSPIRATION (> 10mmHg)

Associated with LARGE PERICARDIAL EFFUSION (insufficient cardiac output caused by inhibition of ventricular filling during diastole)

Question 10

Systolic ejection murmur associated with a systolic click along left upper sternal border

Answer 10

Pulmonary stenosis

Question 11

Round “elf-like” face with full cheeks, flattened bridge of nose, long upper lip, stellate pattern.
Which syndrome?

Answer 11

Williams syndrome.

Question 12

Rib-notching on CXR in asymptomatic child

Answer 12

Coarctation of the aorta
(due to erosion of the inferior portion of the ribs caused by dilated intercostal collateral vessels)

Question 13

Which congenital cardiac defects that can be treated emergently with balloon atrial septostomy?

Answer 13

TGA
Pulmonary atresia
Tricuspid atresia
TAPVR with obstruction at atrial level (i.e. too small PFO)

Question 14

Treatment for significant neurocardiogenic syndrome (aka vasovagal syndrome)?

Answer 14

Fludrocortisone

Question 15

Most common mechanism for SVT in neonate and infants?

Answer 15

Atrioventricular reentry pathway
(not within or near the AV node itself!)

Question 16

Most common mechanism for SVT in older children?

Answer 16

Reentry circuite within or near AV node

Question 17

Treament for persistent ductus arteriosus

Answer 17

NSAID
(ibuprofen, which is preferred, or indomethacin)

Question 18

Systolic murmur that got longer with more prominent click when the patient is standing.
What is the defect?

Answer 18

Mitral valve prolapse

Question 19

Apical systolic murmur with a midsystolic apical click

Answer 19

Mitral valve prolapse

Question 20

What are 2 types of patients where mitral valve prolapse are common?

Answer 20

• Marfan/Ehler-Danlos syndrome
• Anorexia nervosa

Question 21

What are the 2 valves most commonly affected in acute rheumatic fever?

Answer 21

Mitral and aortic valves

Question 22

What are the 5 major criteria for rheumatic fever?

Answer 22

1) carditis (valvular disease with or without myocarditis or pericarditis)
2) erythema marginatum (nonpruritic erythematous, serpiginous, macular lesions with pale centers)
3) subcutaneous nodules (small, painless swellings)
4) polyarthritis (warm, tender, swollen large joints)
5) chorea

Question 23

First line treatment for prolonged QT syndrome in infants

Answer 23

Beta-blocker (propanolol)

Question 24

Low-grade fever, pericarditis, positive ANA, hematosplenomegaly
Toxicity from which drug?

Answer 24

Procainamide

Question 25

What drugs can induce lupus? (2)

Answer 25

Procainamide (no longer on market in the US)
Hydralazine

Question 26

Most common organisms cause infective endocarditis? (3)

Answer 26

1) Staph aureus (more common in children without heart disease)
2) Viridans group strep (more common in children with heart disease)
3) Coagulase-native staph

Question 27

How to evaluate / screen for development of hypertrophic cardiomyopathy in patient with family history?

Answer 27

Genetic testing
(serial ECHO is no longer the right answer)