Long-Term Outcomes After Atrial Switch Operation for Transposition of the Great Arteries

Craig S. Broberg*, Alexandra van Dissel, Jessica Minnier, Jamil Aboulhosn, Robert M. Kauling, Salil Ginde, Eric V. Krieger, Fred Rodriguez, Tripti Gupta, Sangeeta Shah, Anitha S. John, Timothy Cotts, W. Aaron Kay, Marissa Kuo, Cindy Dwight, Patricia Woods, Jeremy Nicolarsen, Berardo Sarubbi, Flavia Fusco, Petra AntonovaSusan Fernandes, Jasmine Grewal, Jonathan Cramer, Paul Khairy, Pastora Gallego, Clare O'Donnell, Jane Hannah, Mikael Dellborg, Carla P. Rodriguez-Monserrate, Isabelle Vonder Muhll, Stephen Pylypchuk, Anthony Magalski, Frank Han, Adam M. Lubert, Joseph Kay, Elizabeth Yeung, Jolien Roos-Hesselink, David Baker, David S. Celermajer, Luke J. Burchill, William M. Wilson, Joshua Wong, Shelby Kutty, Alexander R. Opotowsky

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

1 Citation (Scopus)


Background: For patients with d-loop transposition of the great arteries (d-TGA) with a systemic right ventricle after an atrial switch operation, there is a need to identify risks for end-stage heart failure outcomes. Objectives: The authors aimed to determine factors associated with survival in a large cohort of such individuals. Methods: This multicenter, retrospective cohort study included adults with d-TGA and prior atrial switch surgery seen at a congenital heart center. Clinical data from initial and most recent visits were obtained. The composite primary outcome was death, transplantation, or mechanical circulatory support (MCS). Results: From 1,168 patients (38% female, age at first visit 29 ± 7.2 years) during a median 9.2 years of follow-up, 91 (8.8% per 10 person-years) met the outcome (66 deaths, 19 transplantations, 6 MCS). Patients experiencing sudden/arrhythmic death were younger than those dying of other causes (32.6 ± 6.4 years vs 42.4 ± 6.8 years; P < 0.001). There was a long duration between sentinel clinical events and end-stage heart failure. Age, atrial arrhythmia, pacemaker, biventricular enlargement, systolic dysfunction, and tricuspid regurgitation were all associated with the primary outcome. Independent 5-year predictors of primary outcome were prior ventricular arrhythmia, heart failure admission, complex anatomy, QRS duration >120 ms, and severe right ventricle dysfunction based on echocardiography. Conclusions: For most adults with d-TGA after atrial switch, progress to end-stage heart failure or death is slow. A simplified prediction score for 5-year adverse outcome is derived to help identify those at greatest risk.

Original languageEnglish
Pages (from-to)951-963
Number of pages13
JournalJournal of the American College of Cardiology
Issue number10
Publication statusPublished - 6 Sep 2022

Bibliographical note

Funding Information:
The authors gratefully acknowledge the work of Beth Wilson (Portland, Oregon, USA), Amanda Sammons (Cincinnati, Ohio, USA), Melissa Major (Palo Alto, California, USA), Mikyla Janzen (Vancouver, BC, Canada), Sandra Jaidzeka (Washington, DC, USA), and Mary Stumpf (Indianapolis, Indiana, USA), for their assistance in research coordination. In addition, the authors appreciate Dr Michael Earing, University of Chicago Medicine and Comer Children's Hospital, Chicago, Illinois, USA, for his contribution to the overall study design, execution, and interpretation.

Funding Information:
This study was funded by a joint grant from the Children’s Heart Foundation and the American Heart Association (17GRNT33670334). Dr Opotowsky was supported by the Heart Institute Research Core at Cincinnati Children’s Hospital and the Dunlevie Family Fund. The authors used the Research Electronic Data Capture (REDCap) online database, which is funded by the National Institutes of Health (UL1TR002369). All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Publisher Copyright:
© 2022 American College of Cardiology Foundation


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