The physiology of the Fontan circulation

The Fontan circulation, no matter which of its various iterations, is abnormal in virtually every aspect of its performance. Some of these abnormalities are primarily the result of the procedure itself, and others are secondary to the fundamental disturbances of circulatory performance imposed by the ‘single’ ventricle circulation. The physiology of ventricular, systemic arterial and venopulmonary function will be described in this review.