Diagnosis of Diastolic Dysfunction in Adults With Failing Fontan Circulation

Abstract

Since the initial Fontan procedure introduced in 1968 for tricuspid atresia, significant advancements have expanded its application to various congenital cardiac anomalies where a biventricular circulation is unattainable. Despite improved survival rates, Fontan circulation tends to fail over time leading to late morbidity and mortality. Diastolic dysfunction is increasingly recognized as a significant contributor to circulatory insufficiency and failure in Fontan patients. This review aims to assess the current evidence for diagnosing diastolic dysfunction in adults with failing Fontan circulation, including biomarkers, echocardiography, cardiac magnetic resonance imaging, and catheterization. While advancements have been made in understanding diastolic dysfunction in single ventricles, challenges remain due to the unique anatomy and physiology of Fontan patients. Future research should focus on refining diagnostic parameters and exploring potential therapies tailored to the distinct needs of this population.