Heart Failure in Adults who had the Fontan Procedure: Natural History, Evaluation, and Management.

Fontan type palliations are a group of operations employed in patients with only one functional ventricle. These procedures take the normally parallel pulmonary and systemic circulations and place them in series to reestablish separation between the two. The Fontan circulation established by such operations is a unique, artificial physiologic entity. Although the Fontan circulation supports patients for decades, patients eventually experience progressive heart failure. As this growing group of patients reaches adulthood, there is a need for general cardiologists as well as adult congenital heart disease specialists to be familiar with the long-term sequelae of the Fontan circulation. The purpose of the present review is to summarize the limited available data on heart failure of the Fontan, its natural history, evaluation, and management.