Venous Shunts and the Fontan Circulation in Adult Congenital Heart Disease

Surgical innovation has allowed the majority of patients with functionally univentricular hearts to survive to adulthood. The history of surgical techniques for this form of congenital heart disease is reviewed, together with a summary of the unique cardiovascular physiology which affects multiple body organs. Sequelae of this circulation are reviewed, with focused discussion on the management of late atrial arrhythmias. The importance of lifestyle management, including daily physical activity and maintenance of normal body weight as therapeutic interventions, is emphasised.