Challenge of Hypoplastic Right Ventricle with Multiple Associated Anomalies: Biventricular Repair or Univentricular Approach?

Hypoplastic right ventricle is a rare congenital heart disease, as attested to by the small number of cases reported so far. We present a patient with hypoplastic right ventricle and multiple associated anomalies, namely ventricular septal defect, atrial septal defect, and pulmonary stenosis. Our principal concern was whether biventricular repair or Fontan type surgery would be the optimal management in this patient. We succeeded in performing biventricular repair via multi-modality cardiac imaging using intraoperative transesophageal echocardiography.