Congenital Heart Disease Questions - Set 3¶

Question 1¶

What is the most appropriate initial imaging modality to evaluate suspected vascular ring?

A) Chest X-ray B) Echocardiogram C) CT angiography D) MRI E) Cardiac catheterization

Answer

**Correct Answer: C** **CT angiography** is the gold standard for vascular ring evaluation: - Defines vascular anatomy precisely - Shows airway compression - Quick acquisition (important in children) - 3D reconstruction for surgical planning Echo can miss arch sidedness and ring anatomy. **Board Pearl:** Vascular ring workup = CT angiography (defines anatomy, shows compression)

Question 2¶

A neonate with critical pulmonary stenosis has ductal-dependent pulmonary blood flow. After starting PGE1, what intervention is indicated?

A) Surgical valvotomy B) Balloon pulmonary valvuloplasty C) BT shunt placement D) Observation until hemodynamically stable E) Arterial switch operation

Answer

**Correct Answer: B** **Balloon pulmonary valvuloplasty** is first-line for critical PS: - High success rate (>90%) - Low complication rate - Avoids surgery - Can be done in cath lab once stable on PGE1 **Board Pearl:** Critical PS = balloon valvuloplasty (first-line intervention)

Question 3¶

In scimitar syndrome, which pulmonary vein drains anomalously and where?

A) Right upper pulmonary vein to SVC B) Right pulmonary veins to IVC C) Left pulmonary veins to coronary sinus D) All pulmonary veins to RA E) Right lower pulmonary vein to hepatic vein

Answer

**Correct Answer: B** **Scimitar syndrome**: - Right pulmonary veins drain to IVC - Curved venous course looks like scimitar sword on CXR - Associated with right lung hypoplasia - Systemic arterial supply to right lung common **Board Pearl:** Scimitar = right pulmonary veins to IVC; scimitar sign on CXR

Question 4¶

A patient with L-TGA (congenitally corrected TGA) is at risk for what conduction abnormality?

A) Wolff-Parkinson-White syndrome B) Progressive complete heart block C) Atrial flutter D) Long QT syndrome E) Brugada syndrome

Answer

**Correct Answer: B** L-TGA has progressive AV block: - Abnormal position of AV node - Risk of spontaneous CHB: ~2% per year - May present years after "incidental" diagnosis - Pacemaker often eventually needed **Board Pearl:** L-TGA = progressive CHB (~2%/year); abnormal AV node position

Question 5¶

What is the expected SpO2 in a neonate with obstructed supracardiac TAPVR?

A) 100% B) 85-95% C) 70-80% D) 55-70% E) Variable depending on PDA

Answer

**Correct Answer: D** Obstructed TAPVR: - Pulmonary venous obstruction → pulmonary edema - Very limited blood reaching LA - Severely reduced systemic saturation (55-70%) - **Surgical emergency** **Board Pearl:** Obstructed TAPVR = low SpO2, pulmonary edema, surgical emergency

Question 6¶

Which genetic syndrome is most associated with conotruncal cardiac defects?

A) Down syndrome (Trisomy 21) B) Turner syndrome (45,X) C) 22q11.2 deletion syndrome D) Williams syndrome E) Noonan syndrome

Answer

**Correct Answer: C** **22q11.2 deletion** = conotruncal defects: - Interrupted aortic arch (Type B especially) - Truncus arteriosus - TOF - VSD (conoventricular) The neural crest migration abnormality affects outflow tract development. **Board Pearl:** Conotruncal defects = think 22q11.2 deletion

Question 7¶

A premature infant with a large PDA has diastolic flow reversal in the descending aorta. What does this indicate?

A) Pulmonary hypertension B) Hemodynamically significant PDA C) Impending PDA closure D) Need for surgical ligation E) Coarctation of aorta

Answer

**Correct Answer: B** Diastolic flow reversal = **"ductal steal"**: - Blood flows retrograde during diastole - Systemic end-organs hypoperfused - Indicates hemodynamically significant PDA - Associated with NEC, renal dysfunction, IVH risk **Board Pearl:** Diastolic flow reversal = ductal steal = hemodynamically significant PDA

Question 8¶

What is the natural history of a small perimembranous VSD with septal aneurysm tissue?

A) Will definitely require surgery B) High likelihood of spontaneous closure C) Will develop aortic regurgitation D) Will develop subaortic stenosis E) Stable, but never closes

Answer

**Correct Answer: B** Septal aneurysm tissue = **tricuspid valve tissue partially occluding VSD**: - Sign of spontaneous closure in progress - Very high rate of complete closure - Rarely needs intervention **Board Pearl:** VSD + septal aneurysm = likely to close spontaneously

Question 9¶

A child with aortic stenosis undergoes Ross procedure. What valve is used to replace the aortic valve?

A) Mechanical valve B) Bioprosthetic valve C) Cadaveric homograft D) Patient's own pulmonary valve (autograft) E) Bovine pericardial valve

Answer

**Correct Answer: D** **Ross procedure**: - Native pulmonary valve moved to aortic position (autograft) - Pulmonary position replaced with homograft - Autograft grows with child - No anticoagulation needed - But creates "two-valve disease" **Board Pearl:** Ross = pulmonary autograft to aortic position; grows with child

Question 10¶

In heterotaxy with right atrial isomerism, what splenic finding is expected?

A) Normal spleen B) Multiple spleens (polysplenia) C) Absent spleen (asplenia) D) Accessory spleen only E) Enlarged spleen

Answer

**Correct Answer: C** Right atrial isomerism = **asplenia syndrome**: - Bilateral right-sidedness - No spleen → immunodeficiency - Complex CHD (common AV canal, TAPVR) - Midline liver - Howell-Jolly bodies on blood smear **Board Pearl:** Right isomerism = asplenia; Left isomerism = polysplenia