Background: To date conflicting studies exist regarding differing morphological variants of hypoplastic left heart syndrome and their impact on mortality. Although Aortic Atresia (AA) is identified by some as a risk factor for mortality, recent data suggests that mitral valve patency is important within this context.
Methods: 222 consecutive patients who underwent the Norwood procedure from May 2002 to May 2007 are included in this study. All patients in the study had systemic right ventricles and pulmonary blood flow provided by a right ventricle-to-pulmonary artery conduit at stage 1 palliation. The study population included only patients with Hypoplastic Left Heart Syndrome (HLHS) and were divided into three groups: aortic atresia-mitral atresia (AA-MA), aortic stenosis-mitral stenosis (AS-MS), and aortic atresia-mitral stenosis (AA-MS). Statistical analysis was conducted using a 2 tailed Fisher exact test and differences were considered statistically significant when p<0.05.
Results: Of the 222 patients who underwent the Norwood procedure, 212 had a diagnosis of hypoplastic left heart syndrome. Of these 89 had AA-MA, 74 had AS-MS, and 49 had AA-MS. A Kaplan-Meier survival curve is presented below showing no significant survival differences between morphologic groups (p=0.93). Using 30 day mortality, no statistical differences could be demonstrated between either of the morphologic groups.
Conclusions: The current surgical management of Hypoplastic Left Heart Syndrome at Birmingham Childrens Hospital confers equivalent survival advantage to the different morphologic groups seen in this condition.