Univentricular Heart in Adults: Clinical Outcomes, Diagnostic Challenges, and Therapeutic Approaches

Abstract

Univentricular heart is a rare and complex congenital heart defect characterized by the presence of a single functional ventricular chamber. While significant advancements in pediatric surgical interventions have improved the survival of patients with univentricular physiology, a growing number of these individuals are now reaching adulthood. This transition presents a spectrum of unique clinical challenges, including the management of long-term complications such as heart failure, arrhythmias, thromboembolism, and liver dysfunction. The adult univentricular heart population requires a multidisciplinary approach to optimize outcomes, incorporating advanced imaging modalities for accurate anatomical and hemodynamic assessment, and tailored therapeutic strategies. This review discusses the epidemiology, anatomical and physiological considerations, late complications, and current evidence-based management of adult patients with univentricular heart. Furthermore, it explores emerging therapeutic options, including the use of advanced heart failure therapies and the potential for cardiac transplantation. The paper also highlights the importance of transition care programs from pediatric to adult congenital heart disease specialists to improve the continuity of care and long-term prognosis.