A 6-month-old infant with Down syndrome presents with recurrent lower respiratory tract infections, failure to thrive, and a holosystolic murmur loudest at the lower left sternal border. Echocardiography reveals a complete atrioventricular septal defect (AVSD). At what age is surgical repair MOST appropriately planned?
- A As an emergency at the time of diagnosis
- B 3–6 months of age ✓
- C 12–18 months of age
- D After 2 years of age to allow growth
Correct answer: B. 3–6 months of age
Explanation
Complete AVSD (also called endocardial cushion defect) causes a large left-to-right shunt with a high risk of developing irreversible pulmonary hypertension (Eisenmenger syndrome) early, particularly in Down syndrome patients. Early repair at 3–6 months is the current recommendation to prevent pulmonary vascular disease. Delaying until 12–18 months or beyond risks fixed pulmonary hypertension. Emergency surgery is not indicated unless in refractory heart failure unresponsive to medical management.
Reference: Ghai Essential Pediatrics, 10th ed.
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Written and medically reviewed by the StethoPrep medical team.