Mitral stenosis and aortic atresia in hypoplastic left heart syndrome: Survival analysis after stage i palliation

Background: Mitral stenosis with aortic atresia (MS-AA) has been implicated as a risk factor for decreased survival after stage 1 palliation for hypoplastic left heart syndrome. Conflicting results were reported in the literature evaluating the association of anatomic subtypes and mortality. Our objective was to determine whether MS-AA is associated with increased mortality after stage 1 palliation. Methods: Between January 2005 and May 2009 100 consecutive neonates with hypoplastic left heart syndrome underwent stage 1 palliation. Echocardiograms were reviewed for patency of the mitral and aortic valves as well as presence of ventriculocoronary connections (VCC). Patients were divided into (1) mitral and aortic atresia (MA-AA), (2) MS-AA, and (3) mitral and aortic stenosis groups. Survival analysis was performed, and impact of MS-AA and VCC on early and midterm survival was assessed. Results: Of the 100 patients, 31 had MA-AA, 42 had mitral stenosis and aortic stenosis, and 27 had MS-AA (15 with VCC and 12 without). Stage 1 palliation 30-day survival was 90% for the entire cohort and 88.9% for the MS-AA subtype (not significantly different). Six-month survival was 70% overall and 70.4% for MS-AA (not significantly different). When VCC was taken into account, MS-AA with VCC reached 93% survival (versus 50% for MS-AA without VCC; p < 0.01). Conclusions: Stage 1 palliation for hypoplastic left heart syndrome is associated with satisfactory hospital and midterm survival regardless of anatomic subtype. The variants MS-AA and MA-AA are associated with smaller ascending aorta. Mitral stenosis with aortic atresia or VCC did not adversely influence survival.