Long-term issues after the Fontan procedure

The Fontan procedure is used to treat various serious congenital heart defects. Although many people who have had the procedure live productively into adulthood, as they age, they face several health issues due to the physiology of the Fontan circulation. This article reviews the 4 types of Fontan procedures and the changes caused by the surgery, including single-ventricle physiology, nonpulsatile pulmonary perfusion, systemic venous hypertension, and intracardiac scarring, as well as their sequelae. Key nursing assessment items and possible treatment strategies are reviewed. Additional topics, including pregnancy in patients who have undergone the procedure, infective endocarditis prophylaxis, and health-related quality of life, are briefly discussed. Options for Fontan failure, including Fontan conversion or transplantation, are presented. Potential future solutions are outlined.