Survival After Intervention for Single-Ventricle Heart Disease Over 15 Years at a Single Institution.

Abstract

Children with single-ventricle (SV) heart disease possess a spectrum of heart malformations, yet progress through similar hemodynamic states, suggesting differences in outcomes are related to fundamental morphologic differences, patient characteristics, or procedural pathways. We sought to provide a holistic overview of survival after intervention for SV heart disease at our institution. SV heart disease was defined as patients born with a hypoplastic or dysfunctional ventricle with uncertain or unacceptable candidacy for a 2-ventricle circulation. Patients were stratified into 8 diagnostic groups and 11 procedural categories based on the initial interventional procedure. Between 2005 and 2020, 381 patients born with SV heart disease underwent intervention at our institution. Ten-year survival was highest for patients with double inlet left ventricle (89% ± 7%) and lowest for patients with hypoplastic left heart syndrome (55% ± 5%). Initial palliation with less invasive procedures, such as ductal stent (4-year: 100%) or pulmonary artery banding (10-year: 95% ± 5%), demonstrated superior survival compared with more invasive procedures such as the Norwood procedure (10-year: 59% ± 4%). Survival of patients who achieved a biventricular circulation was superior to patients who remained with SV physiology (10-year: 87% ± 5% vs 63% ± 3%, P = .04). In a multivariable analysis, chromosomal/syndromic abnormality, lower weight, hybrid Norwood procedure, nonleft ventricular dominance, and earlier year of operation were risk factors for death. Survival differences in patients with SV heart disease were related primarily to underlying cardiac anatomy, patient characteristics, and procedural complexity. Left ventricular dominance, more recent intervention, and attainment of a 2-ventricle circulation were associated with improved survival.