Current Results of Single Ventricle Palliation of Patients With Double Inlet Left Ventricle.

BACKGROUND: Double inlet left ventricle (DILV) is a heterogeneous single ventricle anomaly in which initial presentation, and consequently, timing and palliation mode vary based on morphology and degree of pulmonary or systemic outflow obstruction. Very few reports, mostly old, focused on palliation outcomes of DILV. We report current-era results and examine whether morphologic and subsequently surgical factors influence survival.

METHODS: Fifty-eight infants with DILV underwent single ventricle palliation. Echocardiographic examination showed pulmonary (n = 29, 50%), systemic outflow tract (n = 11, 19%), and arch (n = 17, 29%) obstruction. Factors associated with death or transplantation were examined.

RESULTS: Forty-four patients (76%) required neonatal first-stage palliation: modified Blalock-Taussig shunt (n = 15, 26%), Norwood (n = 15, 26%), or pulmonary artery band (n = 14, 24%), whereas 14 (24%) received primary Glenn. There was 1 hospital death (2%) and 2 interstage deaths before Glenn, in addition to 1 late death that was noncardiac. Overall 10-year survival was 94% and was comparable for different palliative surgeries (p = 0.49). Three patients (6%) underwent heart transplantation after first-stage palliation (n = 1) or after Glenn (n = 2) for ventricular noncompaction (n = 1), ventricular and atrioventricular valve dysfunction (n = 1), and pacemaker-induced cardiomyopathy (n = 1). Overall 10-year freedom from death or transplantation was 87% and was comparable for different palliative surgeries (p = 0.58). On regression risk analysis, none of the tested morphologic or surgical variables was associated with the risk of death or transplantation.

CONCLUSIONS: Current outcomes of multistage palliation of DILV are relatively good compared with published reports of other single ventricle anomalies. Survival is not greatly affected by cardiac morphology or initial palliative surgery type.