High-Yield Board Questions¶

Kawasaki Disease¶

Question 1¶

A 4-year-old presents with 6 days of fever, bilateral conjunctival injection, strawberry tongue, cervical lymphadenopathy, and truncal rash. Echo shows LAD diameter of 3.5mm (Z-score +2.8). What is the initial treatment?

A) IVIG 2g/kg alone B) IVIG 2g/kg + high-dose aspirin C) IVIG 2g/kg + high-dose aspirin + corticosteroids D) Infliximab E) Aspirin alone

Answer

**Correct Answer: C** **Explanation:** This patient has complete Kawasaki disease WITH coronary artery aneurysm at diagnosis (Z-score ≥2.5). Per 2024 AHA guidelines, patients with high-risk features (including CAA at diagnosis) should receive **intensified initial therapy**: IVIG + high-dose aspirin + adjunctive corticosteroids. - A is incorrect: Missing aspirin and steroids for high-risk patient - B is incorrect: Missing steroids for high-risk patient - D is incorrect: Infliximab is for IVIG-resistant cases - E is incorrect: IVIG is essential **Board Pearl:** Z-score ≥2.5 at diagnosis = high risk → intensify initial treatment

Question 2¶

In the KAWARACE trial, what was found regarding aspirin dosing in acute Kawasaki disease?

A) High-dose aspirin prevents coronary aneurysms B) High-dose aspirin reduces fever duration significantly C) No significant difference between high-dose and low-dose aspirin D) Low-dose aspirin increases CAA risk E) Aspirin should be avoided in acute phase

Answer

**Correct Answer: C** **Explanation:** The KAWARACE trial (2023) found **no significant difference** between high-dose (30-50 mg/kg/day) and low-dose (3-5 mg/kg/day) aspirin in acute Kawasaki disease. This has led to some centers using low-dose aspirin throughout treatment. **Board Pearl:** KAWARACE challenges traditional high-dose aspirin approach

Hypertrophic Cardiomyopathy¶

Question 3¶

A 16-year-old with HCM has a calculated 5-year SCD risk of 8% using the Norrish pediatric HCM risk model. He has never had syncope and has no family history of sudden death. What is the appropriate recommendation regarding ICD?

A) No ICD indicated - risk too low B) ICD recommended (Class I) C) ICD should be discussed (Class IIa) D) Reassess in 1 year before deciding E) Cardiac transplant evaluation

Answer

**Correct Answer: C** **Explanation:** Per 2024 guidelines, ICD is reasonable (Class IIa) when 5-year SCD risk is ≥6% using validated pediatric models. At 8% 5-year risk, ICD implantation should be discussed with the patient/family as a reasonable option. - Risk <4%: Generally no ICD - Risk 4-6%: Individualize - Risk ≥6%: ICD reasonable (Class IIa) **Board Pearl:** Use Norrish pediatric HCM risk score; ICD discussed at ≥6% 5-year risk

Question 4¶

Which medication has demonstrated myosin inhibition as mechanism of action for treating obstructive HCM?

A) Metoprolol B) Verapamil C) Disopyramide D) Mavacamten E) Diltiazem

Answer

**Correct Answer: D** **Explanation:** **Mavacamten** is a first-in-class cardiac myosin inhibitor that reduces hypercontractility in HCM. The EXPLORER-HCM trial (2020) showed significant reduction in LVOT gradient and symptom improvement. - A, B, E: Negative inotropes through different mechanisms - C: Class Ia antiarrhythmic with negative inotropic effect **Board Pearl:** Mavacamten = cardiac myosin inhibitor; EXPLORER-HCM trial

Channelopathies¶

Question 5¶

A 14-year-old female has genetically confirmed LQTS Type 2 (KCNH2 mutation). Her QTc is 510ms. She is asymptomatic. What is the recommended treatment?

A) No treatment needed if asymptomatic B) Beta-blocker only C) ICD primary prevention D) Beta-blocker + consider ICD discussion E) Mexiletine

Answer

**Correct Answer: D** **Explanation:** This patient has multiple risk factors: - LQT2 (higher risk genotype, especially in females) - QTc >500ms - Female sex (higher risk in LQT2) Beta-blocker therapy is indicated. Given QTc >500 and LQT2 in female, ICD discussion for primary prevention is appropriate (Class IIa indication). - A is incorrect: All LQTS patients need treatment - B is incomplete: QTc >500 warrants ICD discussion - C is incorrect: Secondary prevention reserved for survivors of arrest - E is incorrect: Mexiletine is adjunctive for LQT3 **Board Pearl:** LQT2 female + QTc >500 = higher risk; ICD discussion warranted

Syncope¶

Question 6¶

Which feature MOST strongly suggests cardiac (vs. vasovagal) etiology in a teenager with syncope?

A) Occurred after standing for 30 minutes B) Associated nausea and diaphoresis before event C) Occurred while playing basketball D) Had brief jerking movements after collapse E) Full recovery within 2 minutes

Answer

**Correct Answer: C** **Explanation:** **Exertional syncope** (syncope DURING exercise) is a major red flag for cardiac etiology (HCM, LQTS, CPVT, AAOCA) and mandates full cardiac evaluation. - A: Classic vasovagal trigger - B: Classic vasovagal prodrome - D: Convulsive syncope is common with any prolonged cerebral hypoperfusion - E: Rapid recovery is nonspecific **Board Pearl:** Syncope DURING exercise = cardiac until proven otherwise

Pulmonary Hypertension¶

Question 7¶

A 3-year-old with a large VSD has mean PA pressure of 55 mmHg and PVR of 9 Wood units. Vasoreactivity testing shows no significant response. What is the recommendation?

A) Proceed with VSD closure B) VSD closure is contraindicated C) Treat with sildenafil for 6 months then reassess D) Fenestrated VSD closure E) Heart-lung transplant

Answer

**Correct Answer: B** **Explanation:** With PVR of 9 WU (above the 6-8 WU threshold) and no vasoreactivity, the patient has **irreversible pulmonary vascular disease**. VSD closure would result in RV failure (can't pump against fixed elevated PVR without the "pop-off" of the VSD shunt). This patient likely has or is developing Eisenmenger physiology. - A is incorrect: PVR too high - C/D: No evidence that medical therapy will make this operable - E: May eventually be needed **Board Pearl:** PVR >6-8 WU without vasoreactivity = inoperable

CCHD Screening¶

Question 8¶

A newborn at 28 hours of life has the following CCHD screen: Right hand 97%, Right foot 93%, difference 4%. After 1 hour retest: Right hand 96%, Right foot 92%, difference 4%. Per 2025 guidelines, what is the next step?

A) Pass - discharge with routine follow-up B) Pass after one more retest in 1 hour C) Fail - obtain echocardiogram D) Indeterminate - repeat tomorrow E) Fail - immediate cardiology consult without echo

Answer

**Correct Answer: C** **Explanation:** Per 2025 algorithm: - Initial: 93% (90-94%) and 4% difference (>3%) → RETEST - After 1 retest: Still 92% and 4% difference → **FAIL** 2025 update: **Only 1 retest allowed** (previously 2). After failing the retest, echocardiogram is indicated. **Board Pearl:** 2025 update - only 1 retest; then PASS or FAIL

Fontan¶

Question 9¶

A 15-year-old Fontan patient presents with ascites and lower extremity edema. Albumin is 1.9 g/dL. Stool alpha-1 antitrypsin is elevated. What is the diagnosis?

A) Fontan failure B) Protein-losing enteropathy C) Fontan-associated liver disease D) Right heart failure E) Nephrotic syndrome

Answer

**Correct Answer: B** **Explanation:** **Protein-losing enteropathy (PLE)** is characterized by: - Hypoalbuminemia - Edema/ascites - Elevated stool alpha-1 antitrypsin (confirms enteric protein loss) - Often with hypogammaglobulinemia and lymphopenia This occurs in 5-15% of Fontan patients due to elevated CVP and intestinal lymphatic congestion. **Board Pearl:** PLE = hypoalbuminemia + elevated stool α1-antitrypsin in Fontan

Sports Cardiology¶

Question 10¶

Per 2025 guidelines, which patient with known cardiac condition MAY participate in competitive sports using shared decision-making?

A) 16-year-old with AAOLCA (interarterial course) B) 15-year-old with LQTS on beta-blocker, asymptomatic C) 14-year-old with CPVT on beta-blocker D) 17-year-old with Eisenmenger syndrome E) 18-year-old with severe aortic stenosis

Answer

**Correct Answer: B** **Explanation:** 2025 guidelines allow **shared decision-making** for several conditions including asymptomatic LQTS on appropriate therapy. - A: AAOLCA requires surgical repair before sports - C: CPVT remains restricted from competitive sports - D: Eisenmenger precludes competitive sports - E: Severe AS precludes competitive sports **Board Pearl:** LQTS can participate with shared decision-making (beta-blocker, AED access)

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