Richardson JN; DeBakey Heart & Vascular Center, Houston, Texas, USA.
Martin CM

Methodist DeBakey cardiovascular journal. 22(3):124-135, 2026.

Advances in the management of congenital heart disease have resulted in a
rapidly expanding population of adults with congenital heart disease
(ACHD), among whom heart failure (HF) has emerged as a leading cause of
morbidity and mortality. HF in ACHD represents a distinct and
heterogeneous clinical entity shaped by lifelong abnormal loading
conditions, prior surgical interventions, arrhythmogenic substrates, and
limited representation in randomized clinical trials. The systemic
ventricle may be morphologically left, right, or single, each conferring
unique susceptibility to maladaptive remodeling, myocardial fibrosis,
valvular dysfunction, and progressive contractile decline. Accurate
diagnosis requires longitudinal, multimodal assessment incorporating
echocardiography, cardiovascular magnetic resonance, cardiopulmonary
exercise testing, biomarkers, rhythm surveillance, and selective invasive
hemodynamic evaluation. Management prioritizes identification and
correction of reversible contributors, including residual structural
lesions, atrioventricular valve regurgitation, arrhythmias, pulmonary
vascular disease, and extracardiac comorbidities. Pharmacologic therapy
remains largely extrapolated from acquired HF paradigms and demonstrates
variable efficacy across ACHD subgroups, underscoring the need for
physiology-driven individualized care within specialized centers. Advanced
therapies, including heart transplantation and mechanical circulatory
support, are increasingly utilized, with improving outcomes despite higher
perioperative complexity. This review presents a ventricle-based framework
for understanding the pathophysiology, evaluation, and management of HF in
ACHD and highlights critical gaps requiring further investigation.