Cardiac surgery is consulted on a newborn with a large ventricular septal defect. The child has poor weight gain and feeding difficulties. He requires furosemide and captopril to avoid dyspnea. On physical examination his temperature is 36.9°C (98.4°F), pulse rate is 158/min, respiratory rate is 30/min, and blood pressure is 94/62 mm Hg. Chest auscultation reveals a holosystolic murmur along the left lower sternal border and a mid-diastolic low-pitched rumble at the apex. Abdominal examination reveals the presence of hepatomegaly. An echocardiogram confirms a diagnosis of a membranous VSD while hemodynamic studies show a Qp:Qs ratio of 2.8:1. Which of the following is the best management option?

Continue medical treatment and provide reassurance about spontaneous closure of the defect

Hybrid surgery using both transcatheter device and surgical repair

Transcatheter occlusion of the defect

Addition of digoxin to the current medical regimen with regular follow-up until spontaneous closure occurs

Over the course of a year, 5 children with identical congenital heart defects were referred to a pediatric cardiac surgeon for evaluation. All 5 children had stable vital signs and were on appropriate medication. Upon review of medical records, all of them had a loud holosystolic murmur over the third intercostal space at the left sternal border. The surgeon ordered echocardiograms for all 5 children and recommended surgical closure of the defect in one of them. Which of the following patients required surgical repair of their defect?

A 2-year-old boy with a 2-mm supracristal defect, without heart failure, pulmonary hypertension, or growth failure

A 7-year-old boy with an 11-mm muscular defect and severe pulmonary vascular disease non-responsive to pulmonary vasodilators

An 11-month-old female infant with a 6-mm membranous defect, without heart failure, pulmonary hypertension, or growth failure

A 4-month-old male infant with a 12-mm muscular defect, without heart failure, pulmonary hypertension or growth failure

A 5-year-old girl with a 2-mm membranous defect, no heart failure, a Qp:Qs ratio less than 2:1, and no growth failure

A 1-day-old infant in the general care nursery, born at full term by uncomplicated cesarean section delivery, is noted to have a murmur, but otherwise appears well. On examination, respiratory rate is 40/min and pulse oximetry is 96%. Precordium is normoactive. With auscultation, S1 is normal, S2 is single, and a 2/6 systolic ejection murmur is heard at the left upper sternal border. Echocardiography shows infundibular pulmonary stenosis, overriding aorta, ventricular septal defect and concentric right ventricular hypertrophy. Which of the following correlate with the presence or absence of cyanosis in this baby?

The degree of right ventricular outflow tract obstruction

The ratio of reduced hemoglobin to oxyhemoglobin

The concentration of hemoglobin

The size of ventricular septal defect

The concentration of pulmonary surfactant

A previously healthy 64-year-old man comes to the physician 3 days after noticing a lump in his right groin while he was bathing. He has no history of trauma to the region. He has hypercholesterolemia and hypertension treated with atorvastatin and labetalol. He has smoked 2 packs of cigarettes daily for 35 years. His vital signs are within normal limits. Examination shows a 4-cm (1.6-in), nontender, pulsatile mass with a palpable thrill at the right midinguinal point. On auscultation, a harsh continuous murmur is heard over the mass. Femoral and pedal pulses are palpable bilaterally. The remainder of the examination shows no abnormalities. Which of the following is the most likely diagnosis?

Arteriovenous fistula of the femoral vessels

Congenital heart defects requiring surgery for USMLE Step 2 CK: High-Yield Surgery Lessons

💔 Core concept - Heart's First Flaws

Acyanotic (L→R shunt): ↑ pulmonary blood flow, causing pulmonary HTN. Examples: VSD, ASD, PDA.
Cyanotic (R→L shunt): Deoxygenated blood enters systemic circulation, causing early cyanosis.
- 📌 5 T's: Truncus arteriosus, Transposition, Tricuspid atresia, Tetralogy of Fallot, TAPVR.

⭐ Eisenmenger Syndrome: A severe, late complication where an uncorrected L→R shunt leads to irreversible pulmonary HTN, causing shunt reversal to R→L, resulting in cyanosis.

🔄 Pathophysiology - Shunts & Switches

Left-to-Right (L→R) Shunts (Acyanotic):
- Examples: VSD, ASD, PDA.
- Patho: Oxygenated blood shunts from high-pressure left heart to low-pressure right heart.
- Effect: ↑ Pulmonary blood flow ($Q_p$) → pulmonary HTN, RV hypertrophy.
- 📌 Shunt magnitude measured by $Q_p:Q_s$ ratio; >1.5 is significant.
Right-to-Left (R→L) Shunts (Cyanotic):
- Examples: Tetralogy of Fallot, Transposition of Great Arteries (TGA).
- Patho: Deoxygenated blood bypasses lungs, enters systemic circulation → cyanosis.
"Switch" (TGA):
- Aorta from RV, Pulmonary Artery from LV → two parallel circuits.
- Survival requires mixing via a shunt (PDA, ASD, VSD).

⭐ Eisenmenger Syndrome: Irreversible pulmonary HTN causes shunt reversal from L→R to R→L, leading to late-onset cyanosis. A contraindication to shunt closure.

Patent Ductus Arteriosus (PDA) blood flow diagram

🩺 Clinical Manifestations - Huffs, Puffs & Murmurs

Huffs (Pulmonary Overcirculation): Tachypnea, dyspnea, especially with feeding ("infant angina"). Recurrent respiratory infections.
Puffs (Heart Failure): Poor feeding, failure to thrive (FTT), diaphoresis. Hepatomegaly is a key sign in infants.
Murmurs & Sounds:
- VSD: Harsh holosystolic murmur.
- ASD: Wide, fixed split S2.
- PDA: Continuous "machine-like" murmur.
- TOF: Harsh systolic ejection murmur.
- Coarctation: Systolic murmur radiating to the back; brachial-femoral delay.

⭐ In Tetralogy of Fallot, "tet spells" (hypercyanotic episodes) are relieved by squatting or knee-chest position, which ↑ SVR and ↓ R→L shunting.

🩺 Diagnosis - Echoes & X-Ray Clues

Echocardiogram: Gold standard for diagnosis. Defines anatomy, shunts, and pressures.
Chest X-Ray (CXR) Clues:
- ToF: "Boot-shaped" heart; ↓ pulmonary vascular markings.
- TGA: "Egg on a string"; ↑ pulmonary vascular markings.
- TAPVR: "Snowman" sign (supracardiac type).
- Ebstein's Anomaly: Massive cardiomegaly ("box-shaped" heart).

⭐ Echocardiography is the definitive non-invasive diagnostic tool for virtually all congenital heart defects, guiding surgical planning.

Boot-shaped heart in Tetralogy of Fallot on chest X-ray

🩹❤️ Management - The Surgical Fix

Ductal-Dependent Lesions: Maintain patency with Prostaglandin E1 (PGE1) pre-op.
PDA: Indomethacin; surgical ligation or percutaneous coil embolization.
ASD/VSD: Percutaneous device closure (ASD); surgical patch for large VSDs.
Tetralogy of Fallot (ToF): Complete repair (VSD closure, RVOTO relief). Palliative: Blalock-Taussig-Thomas (BTT) shunt.
d-TGA: Arterial switch (Jatene). Palliative: Rashkind procedure (atrial septostomy).
Coarctation: Resection with end-to-end anastomosis; balloon angioplasty.

⭐ The Blalock-Taussig-Thomas (BTT) shunt connects subclavian to pulmonary artery, palliating cyanosis (e.g., ToF) by ↑ pulmonary blood flow.

⚡ Biggest Takeaways

Tetralogy of Fallot is the most common cyanotic defect; look for "tet spells" and a boot-shaped heart.

Transposition of the Great Arteries causes severe neonatal cyanosis; requires PGE1 then an arterial switch.

VSD, the most common CHD, has a holosystolic murmur; large defects risk Eisenmenger syndrome.

ASD presents with a fixed, wide split S2; closure prevents paradoxical emboli.

PDA features a continuous machine-like murmur; close with indomethacin, keep open with PGE1.

Coarctation of the Aorta shows upper extremity hypertension and is linked to Turner syndrome.

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