TOP3: Over 100 Fontan Patients on Ventricular Assist Device Support: An ACTION Update

Abstract

Background: Many Fontan patients will require end-stage heart failure therapy, and an increasing number are requiring systemic ventricular assist device (VAD) support. This report describes the pre-implant characteristics and clinical outcomes of the largest cohort of Fontan VAD patients. Methods: Fontan patients with systemic VAD support were identified from the Advanced Cardiac Therapies Improving Outcomes Network (ACTION) registry. Patient characteristics and outcomes are described (median, interquartile range). Results: Between 1/2012-12/2022, 107 Fontan patients had VAD implanted (age 10, IQR 5-16 years; 27% female), with 69% as bridge to transplant. Implantable continuous devices (IC-VADs) were most common, with Medtronic Heartware HVAD (31%) and Abbott Heartmate 3 (20%), followed by Berlin EXCOR (22%) and paracorporeal continuous devices (20%). At implant, 58% were on >≥2 inotropes, 26% on extracorporeal membrane oxygenation, 41% were intubated, 48% on total parenteral nutrition, and 8% on dialysis; 28% were INTERMACS Profile 1. Over a median 113 (IQR 43-266) days of support, 75% experienced >≥1 adverse event, with infection most common (10.5 per 100 patient-months) followed by bleeding (8.7 per 100 patient-months) and neurologic dysfunction (5.5 per 100 patient-months). Of patients on IC-VADs, 49% were discharged. At 1 year post-VAD implant, 54% were transplanted, 18% died, and 26% were still on support. Conclusion: Despite requiring high level of support (ECMO, intubation, multiple inotropes) at time of implant, an increasing number of Fontan patients are being successfully supported with VADs, with almost half of patients with IC-VADs discharged on support. Though the majority are alive at 1-year post-implant, adverse events remain common.