Enabling effective care, empowering discovery: Tackling the tough questions about advanced heart failure in adult congenital heart disease

Abstract

Many adults with moderate or complex congenital heart disease (CHD) develop chronic heart or circulatory failure syndromes, often becoming clinically apparent in their 30 s, 40 s, or 50 s and carrying a high risk of mortality. Although the pathophysiology of adult congenital heart disease-related heart failure (ACHD-HF) is highly heterogeneous, it is typically characterized by a prolonged pre-symptomatic phase. During this time, chronic compensatory mechanisms mask disease progression, making the severity of the condition difficult to recognize in a timely manner. This clinical challenge is compounded by fragmented care models, limited specialist resources, and persistent knowledge gaps. Consequently, patients are referred late for advanced therapies, contributing to the excess mortality, particularly in the context of transplant.

While it is recognized that excellent outcomes from transplant and mechanical circulatory support options are achievable in adult CHD patients, contemporary selection practices leave the population undertreated. To advance this cause, we convened a meeting at the 2025 International Society for Heart and Lung Transplantation 45th Annual Meeting & Scientific Sessions in Boston, Massachusetts. The meeting brought together more than 35 participants, including adult and pediatric cardiologists, surgeons, advanced practice providers, researchers, and trainees from North America, Europe and Australia. This diverse group shared opinions regarding priorities for research, education and patient advocacy.

A widely supported outcome was the development of an ACHD-HF specific professional community under the umbrella of ISHLT. This entity would serve as a collaborative home for those dedicated to addressing the knowledge gaps in ACHD-HF, advancing care, and improving outcomes for this growing and vulnerable population.