Fontan Circulation

FontanFontan operation (or total cavopulmonary connection, TCPC) is the final palliation for different CHDs with univentricular physiology. It directs the systemic venous return of the lower part of the body to the lung. A number of anatomic and physiologic parameters need to be optimized prior to and/or increase the risk of successful FontanFontan completion. An extracardiac fenestrated FontanFontan is most common. Chronically elevated venous pressures and lower cardiac output in a FontanFontan circulation lead to end-organ consequences and can result in co-morbidities and/or Fontan circulatory failureFontan circulatory failure. Regular surveillance for FontanFontan-related co-morbidities is recommended. The more common non-cardiac co-morbidities include FontanFontan-associated liver disease (FALD), educational challenges, learning disabilities, short stature, and thromboembolic events. Cardiac mechanisms of failure include heart failureHeart failure, cyanosis, atrioventricular valve regurgitation (AVVR), and arrhythmiasArrhythmia. Non-cardiac mechanisms are rare and associated with lymphatic abnormalities including protein-losing enteropathy (PLEPLE) and plastic bronchitis. SurvivalSurvival has continued to improve though progressive multisystem changes challenge longExtracardiac Fontan term survivalSurvival.