A neonate presents with severe cyanosis at birth unresponsive to oxygen supplementation (hyperoxia test remains positive). ECG shows right axis deviation and right ventricular hypertrophy. Chest X-ray shows an 'egg on string' cardiac silhouette with a narrow superior mediastinum. Which of the following is the immediate management while awaiting surgery?
- A Indomethacin infusion to close the ductus
- B Prostaglandin E1 infusion to maintain ductal patency
- C Administration of 100% oxygen to reduce pulmonary vascular resistance
- D Immediate balloon atrial septostomy (Rashkind procedure) ✓
Explanation
The 'egg on string' silhouette with a narrow mediastinum is pathognomonic of transposition of the great arteries (TGA). In TGA, the pulmonary and systemic circulations are in parallel; survival depends on mixing, which occurs through any communication (ASD, VSD, PDA). The immediate life-saving procedure is balloon atrial septostomy (Rashkind procedure) to create or enlarge an ASD and improve inter-circulatory mixing. Prostaglandin E1 is also used to maintain PDA patency as a temporizing measure, but Rashkind is the procedure of choice for urgent stabilization. Definitive surgery is the arterial switch operation (Jatene). Indomethacin would close the ductus and worsen cyanosis.
Reference: Ghai Essential Pediatrics, 10th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.