title: D-transposition of the great arteries: the current era of the arterial switch operation.
abstract: This paper aims to update clinicians on "hot topics" in the management of patients with D-loop transposition of the great arteries (D-TGA) in the current surgical era. The arterial switch operation (ASO) has replaced atrial switch procedures for D-TGA, and 90% of patients now reach adulthood. The Adult Congenital and Pediatric Cardiology Council of the American College of Cardiology assembled a team of experts to summarize current knowledge on genetics, pre-natal diagnosis, surgical timing, balloon atrial septostomy, prostaglandin E1 therapy, intraoperative techniques, imaging, coronary obstruction, arrhythmias, sudden death, neoaortic regurgitation and dilation, neurodevelopmental (ND) issues, and lifelong care of D-TGA patients. In simple D-TGA: 1) familial recurrence risk is low; 2) children diagnosed pre-natally have improved cognitive skills compared with those diagnosed post-natally; 3) echocardiography helps to identify risk factors; 4) routine use of BAS and prostaglandin E1 may not be indicated in all cases; 5) early ASO improves outcomes and reduces costs with a low mortality; 6) single or intramural coronary arteries remain risk factors; 7) post-ASO arrhythmias and cardiac dysfunction should raise suspicion of coronary insufficiency; 8) coronary insufficiency and arrhythmias are rare but are associated with sudden death; 9) early- and late-onset ND abnormalities are common; 10) aortic regurgitation and aortic root dilation are well tolerated; and 11) the aging ASO patient may benefit from "exercise-prescription" rather than restriction. Significant strides have been made in understanding risk factors for cardiac, ND, and other important clinical outcomes after ASO.
authors.affiliation: Department of Pediatrics (Cardiology), University of Kentucky, Lexington, Kentucky. Electronic address: juanvillaf@yahoo.com.
authors.forename: Juan
authors.initials: J
authors.lastname: Villafañe
authors.affiliation: Pediatric Cardiology, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas.
authors.forename: M Regina
authors.initials: MR
authors.lastname: Lantin-Hermoso
authors.affiliation: Adult Congenital Heart Disease Program, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts.
authors.forename: Ami B
authors.initials: AB
authors.lastname: Bhatt
authors.affiliation: Cardiothoracic Surgery, Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, Wisconsin.
authors.forename: James S
authors.initials: JS
authors.lastname: Tweddell
authors.affiliation: Department of Cardiology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.
authors.forename: Tal
authors.initials: T
authors.lastname: Geva
authors.affiliation: Department of Cardiac Surgery, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.
authors.forename: Meena
authors.initials: M
authors.lastname: Nathan
authors.affiliation: Department of Pediatric Cardiothoracic Surgery, The Great Ormond Street Hospital for Children, NHS Foundation Trust, London, United Kingdom.
authors.forename: Martin J
authors.initials: MJ
authors.lastname: Elliott
authors.affiliation: Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: Victoria L
authors.initials: VL
authors.lastname: Vetter
authors.affiliation: Department of Exercise Physiology, Perlman School of Medicine, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania.
authors.forename: Stephen M
authors.initials: SM
authors.lastname: Paridon
authors.affiliation: University of Minnesota Children's Hospital, Minneapolis, Minnesota.
authors.forename: Lazaros
authors.initials: L
authors.lastname: Kochilas
authors.affiliation: Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.
authors.forename: Kathy J
authors.initials: KJ
authors.lastname: Jenkins
authors.affiliation: Division of Cardiology, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, Ohio.
authors.forename: Robert H
authors.initials: RH
authors.lastname: Beekman
authors.affiliation: The Heart Program, Miami Children's Hospital, Florida International University Herbert Wertheim College of Medicine, Miami, Florida.
authors.forename: Gil
authors.initials: G
authors.lastname: Wernovsky
authors.affiliation: The Heart Institute, Division of Cardiology, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, Ohio.
authors.forename: Jeffrey A
authors.initials: JA
authors.lastname: Towbin
mesh_terms: D006348:Cardiac Surgical Procedures / Q000379:methods*; D006801:Humans; D014188:Transposition of Great Vessels / Q000601:surgery; D014656:Vascular Surgical Procedures / Q000379:methods*
keywords: adult congenital heart disease; aortic dilation; congenital heart defect; congenital heart surgery and sequelae; coronary insufficiency; prostaglandin E
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