Characteristics and Outcomes of Double Outlet Left Ventricle

BACKGROUND: Double outlet left ventricle (DOLV) is a rare cardiac malformation in which both great arteries originate from the morphological left ventricle. There are limited data regarding the anatomical spectrum, characteristics and outcomes of DOLV. METHODS: All patients with DOLV between 1960 and 2008 were identified. Patients' presentation, anatomy, surgical history, and outcomes were recorded. Kaplan-Meier estimates were used to describe time-related survival and freedom from surgical intervention. RESULTS: There were 19 patients with DOLV during the study period, 14 of whom were male (74%). Median age at presentation was 2 days (birth--5.5 years). The most prominent associated cardiac morphologies (in addition to DOLV) were atrial septal defects (14, 74%), ventricular septal defects (13, 68%), right ventricular hypoplasia (12, 63%), and patent ductus arteriosus (15, 80%). At presentation, nine patients (47%) were cyanosed, nine (42%) had cardiomegaly, and all had some degree of right ventricular dysfunction. Of the 19 patients, seven underwent biventricular repairs, nine underwent univentricular palliations, one had only palliative care, and two were seen for consultation only. There were six deaths in this series, five were cardiac in nature (one due to genetic syndrome). Five-year survival after presentation for both biventricular and univentricular repairs was 70-75% (P= 0.80). Most surviving patients had residual cardiac abnormalities at last follow-up despite surgical repair. DISCUSSION: DOLV is a rare cardiac malformation associated with high mortality and guarded outcomes from residual lesions.