Biventricular repair of hypoplastic right ventricle assisted by pulsatile bidirectional cavopulmonary anastomosis

The right ventricle in patients with severe outflow obstruction or atresia and a small tricuspid valve often remains too hypoplastic even after optimal palliation to tolerate biventricular repair with closure of the atrial septal defect. In these patients, nonpulsatile cavopulmonary (Glenn) anastomosis has traditionally facilitated biventricular repair. In 1989, Billingsley and associates reported the addition of a bidirectional cavopulmonary anastomosis to the definitive biventricular repair in patients with hypoplastic right ventricle, pulmonary atresia, and intact ventricular septum. The atrial septal defect was left open with an adjustable snare for later closure. We report five patients with hypoplastic right ventricle (mean diastolic volume 48.4%, mean stroke volume 40.2% of predicted value) who had the atrial septal defect closed at the time of the biventricular repair. Four patients, who had the bidirectional cavopulmonary anastomosis supplementing the biventricular repair, had no evidence of excessive right atrial or superior vena cava hypertension postoperatively. One patient, who had atypical tetralogy of Fallot with tricuspid stenosis, developed recurrent pericardial tamponade and marked hepatomegaly following conventional tetralogy repair with closure of the atrial septal defect. These complications were controlled with the addition of bidirectional cavopulmonary anastomosis 2 months later. Postoperative hemodynamic or Doppler studies in these patients revealed pulsatile flow in the entire pulmonary artery system, including the artery distal to the Glenn anastomosis. This modification of biventricular repair allows primary closure of the atrial septal defect and provides pulsatile arterial flow in the entire pulmonary artery, even when the right ventricle is significantly hypoplastic.