Current management of double-outlet left ventricle

BACKGROUND: Double-outlet left ventricle (DOLV) is an extremely rare type of ventriculoarterial connection, mostly reported anecdotally with wide variations in morphology, surgical strategy and clinical outcome. The aim of the present study was to define current morbidity and mortality of biventricular repair and discuss surgical management in infancy. METHODS: Inclusion criteria were English literature publication; balanced ventricular development and completion of biventricular repair. Institutional experience was also reported. Study end points were survival and freedom from cardiovascular events. Univariate analysis to identify risk factors for complications was performed. RESULTS: During a 45-year interval, 37 cases of DOLV were identified. Median age at repair was 48 months (range 0.3-336); seven patients were infants. Extracardiac repair was performed in 21 (57%) patients, right ventricular outflow patch in seven, pulmonary translocation in six, and intraventricular repair in three. There were three (8%) hospital deaths, due to right heart failure, and seven (21%) patients experienced 10 perioperative complications. Univariate analysis showed year of operation (P = 0.02) and outflow patch technique (P = 0.003) as risk factors for hospital morbidity and mortality. During a median follow-up of 26 months (range 1-192), there was one death and seven reoperations. Ten-year survival and freedom from adverse cardiovascular events were 87 ± 6 and 70 ± 10%, respectively. CONCLUSION: Although seldom reported in the past, repair of DOLV in infancy does not increase surgical risk. Early and late morbidity are mostly due to right heart complications and more common after right ventricular outflow patch repair. Paucity of follow-up information may underestimate actual late adverse event rate.