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J Am Coll Cardiol. 2014 Aug 5;64(5):498-511. doi: 10.1016/j.jacc.2014.06.1150.

D-transposition of the great arteries: the current era of the arterial switch operation.

Author information

1
Department of Pediatrics (Cardiology), University of Kentucky, Lexington, Kentucky. Electronic address: juanvillaf@yahoo.com.
2
Pediatric Cardiology, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas.
3
Adult Congenital Heart Disease Program, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts.
4
Cardiothoracic Surgery, Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, Wisconsin.
5
Department of Cardiology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.
6
Department of Cardiac Surgery, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.
7
Department of Pediatric Cardiothoracic Surgery, The Great Ormond Street Hospital for Children, NHS Foundation Trust, London, United Kingdom.
8
Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.
9
Department of Exercise Physiology, Perlman School of Medicine, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania.
10
University of Minnesota Children's Hospital, Minneapolis, Minnesota.
11
Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.
12
Division of Cardiology, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, Ohio.
13
The Heart Program, Miami Children's Hospital, Florida International University Herbert Wertheim College of Medicine, Miami, Florida.
14
The Heart Institute, Division of Cardiology, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, Ohio.

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.

KEYWORDS:

adult congenital heart disease; aortic dilation; congenital heart defect; congenital heart surgery and sequelae; coronary insufficiency; prostaglandin E

PMID:
25082585
PMCID:
PMC4340094
DOI:
10.1016/j.jacc.2014.06.1150
[Indexed for MEDLINE]
Free PMC Article

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