Journal of Pediatric Cardiology and Cardiac Surgery

Pulmonary hypoplasia and atelectasis due to significant cardiomegaly during fetal life can lead to poor ventilation after the Starnes procedure in patients with tricuspid valve hypoplasia or Ebstein disease, making the postoperative management challenging. We report a neonate who had been diagnosed with tricuspid valve dysplasia with severe regurgitation at 21 weeks of gestation. The female baby underwent right ventricular exclusion on the day of her birth. Postoperatively, veno-venous extracorporeal membrane oxygenation support effectively treated severe hypoxia and pulmonary dysfunction.