Single stage biventricular repair of hypoplastic right ventricle with straddling tricuspid valve.

We present the case of a five-year-old child with an inlet ventricular septal defect, subpulmonic stenosis, hypoplastic right ventricle, and straddling tricuspid valve who received a successful one-stage biventricular repair with right ventricular rehabilitation, right ventricular outflow tract augmentation, papillary muscle transposition, ventricular septal defect closure, and fenestrated atrial septation. This report outlines the surgical decision making and operative technique.