Surgical management of double inlet ventricle

Abstract

Surgical experience with the management of patients with double inlet ventricle (DIV) has undergone continued evolution over the past decade. We report our experience with all forms of surgical treatment for this malformation. Systemic‐pulmonary artery shunts were constructed in 55 patients with 1 hospital death (mortality 2%) from 1967 to 1982. The 10‐year actuarial survival was 85%, but onethird of the group required additional operations during the follow‐up period. Good late functional status was achieved from this procedure. Pulmonary artery banding was performed in 11 patients with no hospital mortality and their 10‐year actuarial survival was 74%. Our overall experience with ventricular septation indicates that it remains the best definitive surgical option for those with mild or absent pulmonary stenosis. It is best advised for those with a dominant chamber of left ventricular morphology. The need for concomitant valve replacement, the use of an extracardiac conduit, and less‐than‐moderate ventricular enlargement have been associated with increased hospital mortality. In the absence of these, the hospital mortality rate was 6%. The Fontan‐Kreutzer operation was performed in 73 patients with 16 hospital deaths. Fourteen of these had DIV. A multivariate logistic analysis showed that young age at operation, primary cardiac anomaly other than tricuspid atresia, and complex additional procedures are incremental risk factors associated with hospital mortality. The overall 10‐year actuarial survival was 71%, and there was no difference among those with tricuspid atresia or other anomalies including DIV. This report presents our results and discusses our current management program for patients with this cardiac malformation.