TY - CHAP M1 - Book, Section TI - Tricuspid Atresia and the Functionally Single Ventricle A1 - van Doorn, Carin A. A1 - Tsang, Victor T. A1 - de Leval, Marc R. A2 - Yuh, David D. A2 - Vricella, Luca A. A2 - Yang, Stephen C. A2 - Doty, John R. Y1 - 2014 N1 - T2 - Johns Hopkins Textbook of Cardiothoracic Surgery AB - EpidemiologyTricuspid atresia is the most common type of functionally single ventricle. Associated cardiac abnormalities are always present and may lead to obstruction of pulmonary or systemic outflow.Morphology and pathophysiologyThe pulmonary and systemic circulations are in parallel. The degree of hypoxemia depends on pulmonary blood flow, which is determined by the associated cardiac abnormalities and the pulmonary vascular bed, both of which are subject to change over time.DiagnosisEchocardiography provides detailed structural and functional information on the heart, particularly in infants. Cardiac catheterization, magnetic resonance imaging, and computed tomography may be necessary to gather additional information.TreatmentWithout surgical treatment the majority of patients will die in infancy, either from severe cyanosis or, less commonly, congestive heart failure. The Fontan circulation is widely accepted as the definitive surgical palliation for patients with tricuspid atresia and other anomalies with functionally single ventricles. There are strict selection criteria for the Fontan operation. In particular, preserved ventricular and valvular function and an adequate pulmonary vascular bed are mandatory. Many patients require preliminary operations in infancy to balance pulmonary and systemic circulations. The surgical technique for the Fontan operation has evolved over time.OutcomeOperative mortality for suitable Fontan candidates in the current era is approximately 5 percent. Late attrition of the Fontan circulation is a serious clinical problem, and its mechanisms are poorly understood. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/28 UR - accesssurgery.mhmedical.com/content.aspx?aid=1104597569 ER -