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

Juan Villafañe, M. Regina Lantin-Hermoso, Ami B. Bhatt, James S. Tweddell, Tal Geva, Meena Nathan, Martin J. Elliott, Victoria L. Vetter, Stephen M. Paridon, Lazaros Kochilas, Kathy J. Jenkins, Robert H. Beekman, Gil Wernovsky, Jeffrey A. Towbin

Research output: Contribution to journalReview articlepeer-review

133 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)498-511
Number of pages14
JournalJournal of the American College of Cardiology
Issue number5
StatePublished - Aug 5 2014


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


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