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The Adult Patient with a Fontan. 2020

Ahmed AlZahrani, and Rahul Rathod, and Ahmed Krimly, and Yezan Salam, and AlJuhara Thaar AlMarzoog, and Gruschen R Veldtman
Adult Congenital Heart Disease Program, Paediatric Cardiology, Prince Sultan Cardiac Centre, PO Box 7897 - G352, Riyadh 11159, Saudi Arabia.

The authors summarize the most important anatomic and physiologic substrates of Fontan circulation. Common anatomic substrates include hypoplastic left heart syndrome, tricuspid atresia, double inlet left ventricle, and unbalanced atrioventricular septal defects. After the Fontan operation exercise capacity is limited and the key hemodynamic drivers is limited preload due to a relatively fixed pulmonary vascular resistance. The authors provide contemporary data on survival, morbidity, and need for reintervention. Operative morality is now expected to be less than 1% and 30 year survival approximately 89%. The authors delineate potential therapeutic approaches for the potential late complications.

UI MeSH Term Description Entries
D011183 Postoperative Complications Pathologic processes that affect patients after a surgical procedure. They may or may not be related to the disease for which the surgery was done, and they may or may not be direct results of the surgery. Complication, Postoperative,Complications, Postoperative,Postoperative Complication
D011379 Prognosis A prediction of the probable outcome of a disease based on a individual's condition and the usual course of the disease as seen in similar situations. Prognostic Factor,Prognostic Factors,Factor, Prognostic,Factors, Prognostic,Prognoses
D012086 Reoperation A repeat operation for the same condition in the same patient due to disease progression or recurrence, or as followup to failed previous surgery. Revision, Joint,Revision, Surgical,Surgery, Repeat,Surgical Revision,Repeat Surgery,Revision Surgery,Joint Revision,Revision Surgeries,Surgery, Revision
D006330 Heart Defects, Congenital Developmental abnormalities involving structures of the heart. These defects are present at birth but may be discovered later in life. Congenital Heart Disease,Heart Abnormalities,Abnormality, Heart,Congenital Heart Defect,Congenital Heart Defects,Defects, Congenital Heart,Heart Defect, Congenital,Heart, Malformation Of,Congenital Heart Diseases,Defect, Congenital Heart,Disease, Congenital Heart,Heart Abnormality,Heart Disease, Congenital,Malformation Of Heart,Malformation Of Hearts
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D017079 Exercise Tolerance The exercise capacity of an individual as measured by endurance (maximal exercise duration and/or maximal attained work load) during an EXERCISE TEST. Tolerance, Exercise
D018729 Fontan Procedure A procedure in which total right atrial or total caval blood flow is channeled directly into the pulmonary artery or into a small right ventricle that serves only as a conduit. The principal congenital malformations for which this operation is useful are TRICUSPID ATRESIA and single ventricle with pulmonary stenosis. Fontan Circuit,Fontan Circulation,Fontan Operation,Fontan Palliation,Hemi-Fontan Procedure,Bidirectional Cavopulmonary Shunt,Bidirectional Glenn Procedure,Bidirectional Glenn Shunt,Norwood Procedure, Stage 2,Norwood Procedure, Stage 3,Norwood Procedure, Stage II,Norwood Procedure, Stage III,Stage 2 Norwood Procedure,Stage 3 Norwood Procedure,Stage II Norwood Procedure,Stage III Norwood Procedure,Bidirectional Cavopulmonary Shunts,Bidirectional Glenn Procedures,Bidirectional Glenn Shunts,Cavopulmonary Shunt, Bidirectional,Circuit, Fontan,Circulation, Fontan,Glenn Procedure, Bidirectional,Glenn Shunt, Bidirectional,Hemi Fontan Procedure,Operation, Fontan,Palliation, Fontan,Procedure, Bidirectional Glenn,Procedure, Fontan,Procedure, Hemi-Fontan,Shunt, Bidirectional Cavopulmonary

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