Comprehensive Approach to the Management of Patients With Hypoplastic Left Heart Syndrome: Analysis of 100 Consecutive Neonates.

Abstract

We report our comprehensive approach to the management of patients with hypoplastic left heart syndrome (HLHS) and describe our outcomes in 100 consecutive neonates. We stratified 100 consecutive neonates (January 1, 2015 to September 1, 2023, inclusive) into 3 pathways. Pathway 1: 77 of 100 (77%) were standard risk and underwent an initial Norwood Stage 1. Pathway 2: 10 of 100 (10%) were high-risk with noncardiac risk factors and underwent an initial Hybrid Stage 1. Pathway 3: 13 of 100 (13%) were high-risk with cardiac risk factors: 10 underwent an initial Hybrid Stage 1 + Ventricular Assist Device insertion (HYBRID+VAD), and 3 were supported with prostaglandin as a planned bridge to primary cardiac transplantation. The overall 1-year mortality for the entire cohort of 100 patients was 9% (9 of 100). Pathway 1: Operative Mortality in Pathway 1 for the initial Norwood Stage 1 was 2.6% (2 of 77). Of the 75 survivors of Norwood Stage 1, 72 underwent successful Glenn, 2 underwent successful biventricular repair, and 1 underwent successful cardiac transplantation. Pathway 2: Operative Mortality in Pathway 2 for the initial Hybrid Stage 1 without VAD was 10% (1 of 10). Of 9 survivors of Hybrid Stage 1, 4 underwent successful cardiac transplantation, 1 died while awaiting cardiac transplantation, 3 underwent Comprehensive Stage 2 (with 1 Operative Mortality after Comprehensive Stage 2), and 1 underwent successful biventricular repair. Pathway 3: Of 10 patients supported with initial HYBRID+VAD in Pathway 3, 7 (70%) underwent successful cardiac transplantation and are alive today, and 3 (30%) died on VAD while awaiting transplantation. Median VAD support time was 134 days (range, 56-226 days). Of 3 patients who were bridged to transplant with prostaglandin, 2 underwent successful transplantation and 1 died while awaiting transplantation. A comprehensive approach to the management of patients with HLHS is associated with an Operative Mortality after Norwood of 2.6% (2 of 77) and an overall 1-year mortality of 9% (9 of 100). Ten patients (10%) were stabilized with HYBRID+VAD while awaiting transplantation. VAD facilitates survival on the waiting list during prolonged waiting times.