Rapid Review High-Yield Questions¶

Question 1¶

What is the inheritance pattern of Long QT syndrome types 1, 2, and 3?

A) X-linked recessive B) Autosomal recessive C) Autosomal dominant D) Mitochondrial E) Variable by type

Answer

**Correct Answer: C** Romano-Ward syndrome (LQT1, 2, 3) = **autosomal dominant**: - LQT1: KCNQ1 (potassium channel) - LQT2: KCNH2 (potassium channel) - LQT3: SCN5A (sodium channel) Jervell and Lange-Nielsen = autosomal recessive + deafness **Board Pearl:** Romano-Ward LQTS = autosomal dominant; JLN = autosomal recessive + deafness

Question 2¶

What is the first-line imaging for suspected anomalous left coronary artery from the pulmonary artery (ALCAPA)?

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

Answer

**Correct Answer: B** **Echocardiography** is first-line: - Shows dilated LV with poor function - Can visualize anomalous coronary origin - Color flow in PA (retrograde from RCA) - ECG may show lateral Q waves CTA or cath to confirm before surgery. **Board Pearl:** ALCAPA: echo first; look for retrograde flow in PA

Question 3¶

What medication should be avoided in Brugada syndrome?

A) Propranolol B) Amiodarone C) Flecainide D) Mexiletine E) Isoproterenol

Answer

**Correct Answer: C** **Flecainide** (and all Class IC sodium channel blockers) unmask/worsen Brugada: - Used for provocation testing - Contraindicated for chronic use - See www.brugadadrugs.org for complete list Also avoid: Class IA antiarrhythmics, certain psychotropics, cocaine **Board Pearl:** Brugada = avoid sodium channel blockers (flecainide); see brugadadrugs.org

Question 4¶

What is the most common cardiac tumor in children?

A) Myxoma B) Rhabdomyoma C) Fibroma D) Teratoma E) Hemangioma

Answer

**Correct Answer: B** **Rhabdomyoma** = most common pediatric cardiac tumor: - ~60% of pediatric cardiac tumors - Strong association with tuberous sclerosis (80%+) - Usually regress spontaneously - Intervention only if symptomatic (obstruction, arrhythmias) **Board Pearl:** Rhabdomyoma = most common pediatric cardiac tumor; associated with tuberous sclerosis

Question 5¶

What prostaglandin E1 dose is used to maintain ductal patency in ductal-dependent lesions?

A) 0.01-0.05 mcg/kg/min B) 0.05-0.1 mcg/kg/min C) 0.5-1 mcg/kg/min D) 5-10 mcg/kg/min E) 50-100 mcg/kg/min

Answer

**Correct Answer: B** **PGE1 dosing**: 0.05-0.1 mcg/kg/min: - Can start higher (0.1) then wean to maintenance - Side effects: apnea (10-20%), fever, hypotension - Monitor for apnea - may need intubation **Board Pearl:** PGE1 = 0.05-0.1 mcg/kg/min; watch for apnea