A full-term neonate presents at 5 days of age with sudden cardiovascular collapse. Prior to collapse, the infant fed well and had no cyanosis. On examination: mottled skin, absent femoral pulses, hepatomegaly, severe metabolic acidosis on blood gas. Echocardiography shows absent left ventricular cavity with aortic atresia. Which prostaglandin E1 dose and route is used as a bridge to surgical palliation?
- A PGE1 0.01 mcg/kg/min subcutaneous once ductal patency confirmed
- B PGE1 inhaled 20 ppm to reduce pulmonary hypertension
- C PGE1 0.05–0.1 mcg/kg/min IV to maintain ductal patency ✓
- D PGE2 oral tablet 0.025 mg/kg/day as outpatient ductal management
Explanation
Hypoplastic left heart syndrome (HLHS) is a duct-dependent systemic circulation lesion where survival depends on maintaining patent ductus arteriosus (PDA) for systemic blood flow. PGE1 (alprostadil) is administered intravenously at 0.05–0.1 mcg/kg/min to maintain ductal patency as a bridge to Norwood Stage I palliation or cardiac transplantation. Side effects of PGE1 include apnea (requiring ventilatory support), hypotension, fever, and jitteriness. Inhaled PGE1 would reduce pulmonary vascular resistance — the opposite of what is needed when systemic blood flow depends on ductal right-to-left shunting.
Reference: Ghai Essential Pediatrics, 10th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.