Abstract

Hypoplastic right ventricular is a rare congenital heart disease. A few cases have been reported. We presented a case with hypoplastic right ventricular and multiple associated anomaly (Ventricular septal defect, atrial septal defect and pulmonary stenosis) in whom the main concern was whether biventricular repair or Fontan type surgery would be the optimal management in this patient. Biventricular repair was done successfully using the multimodality cardiac imaging with intraoperative transesophageal echocardiography.