Long-term Results after Bidirectional Glenn Anastomosis in Patients with Hypoplastic Right Heart Syndrome: A Ten-year Single Center Experience

Background: The Bidirectional Glenn (BDG) procedure in patients with hypoplastic right heart pathology has been proposed to improve clinical outcome for a staged Fontan Circulation with total cavopulmonary connection (TCPC). The aim of the study was to evaluate the long-term results after BDG and to determine risk factors which contraindicating final TCPC completion.

Methods: Eighty-two patients (2.5 months to 30 years) underwent a BDG procedure at our institution from April 1999 to November 2010 and were included in this study. All patients selected for BDG had different types of hypoplastic right heart syndrome. A univariate analysis was performed to evaluate predictors of death, surgical take down and contraindicating for TCPC completion.

Results: The 10-year survival rate was 94.74%. There were two in-hospital deaths and no late deaths. Two patients underwent a surgical takedown operation. Fifty-nine patients underwent successful TCPC completion and thirteen patients are currently waiting for TCPC. Completion was contraindicated in two patients and six patients were lost during follow-up.

A univariate analysis revealed that significant predictors of death, takedown and contraindication for TCPC completion included diagnosis of an unbalanced atrioventricular septal defect (AVSD), repair of total anomalous pulmonary venous drainage (TAPVD) in previous surgery, elevated mean pulmonary arterial pressure and prolonged operation time.

Conclusions: The staged strategy with BDG and terminal TCPC in patients with hypoplastic right heart syndrome provides excellent clinical results. However, there are potential risk factors determining early- and long-term survival.