TY - CHAP M1 - Book, Section TI - Single Ventricle Post Fontan Palliation: Tricuspid Atresia, Pulmonary Atresia, and Hypoplastic Left Heart Syndrome A1 - Chaix, Marie-A A1 - Khairy, Paul A2 - Fuster, Valentin A2 - Narula, Jagat A2 - Vaishnava, Prashant A2 - Leon, Martin B. A2 - Callans, David J. A2 - Rumsfeld, John S. A2 - Poppas, Athena PY - 2022 T2 - Fuster and Hurst's The Heart, 15e AB - Chapter SummaryThis chapter describes management of the single ventricle post-Fontan palliation. The Fontan procedure has been used to palliate a broad spectrum of univentricular physiologies when biventricular repair is not feasible. Although Fontan physiology represents a hemodynamic compromise between systemic venous hypertension and pulmonary hypotension, it is generally considered successful if venous congestion is mild, along with the reduction in cardiac output. Failing Fontan is characterized by marked venous congestion and low cardiac output with multiorgan manifestations. A relatively uneventful clinical course during the first 10 to 15 years after Fontan surgery may be followed by the onset of complications such as arrhythmias, heart failure, increased pulmonary vascular resistance, protein losing enteropathy, thromboembolism, and liver disease (see Fuster and Hurst’s Central Illustration). As a result of considerable morbidity in adulthood, regular screening and surveillance with laboratory tests, imaging studies, arrhythmia monitoring, and objective assessment of functional capacity is required by caregivers with expertise in congenital heart disease. The threshold for cardiac catheterization should generally be low in the setting of new-onset or progressive symptoms. Therapeutic management is dictated by the type of complication encountered. In patients refractory to medical therapy and/or percutaneous interventions, surgical options (including Fontan conversion and transplantation) should be discussed by a multidisciplinary team. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/10/11 UR - accessmedicine.mhmedical.com/content.aspx?aid=1202452441 ER -