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 3rd; 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 Antonova; Susan 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

doi:10.1016/j.jacc.2022.06.020

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

J Am Coll Cardiol. 2022 Sep 6;80(10):951-963. doi: 10.1016/j.jacc.2022.06.020.

Authors

Craig S Broberg¹, Alexandra van Dissel², Jessica Minnier³, Jamil Aboulhosn⁴, Robert M Kauling⁵, Salil Ginde⁶, Eric V Krieger⁷, Fred Rodriguez 3rd⁸, Tripti Gupta⁹, Sangeeta Shah⁹, Anitha S John¹⁰, Timothy Cotts¹¹, W Aaron Kay¹², Marissa Kuo⁹, Cindy Dwight¹², Patricia Woods¹³, Jeremy Nicolarsen¹⁴, Berardo Sarubbi¹⁵, Flavia Fusco¹⁵, Petra Antonova¹⁶, Susan Fernandes¹⁷, Jasmine Grewal¹⁸, Jonathan Cramer¹⁹, Paul Khairy²⁰, Pastora Gallego²¹, Clare O'Donnell²², Jane Hannah²², Mikael Dellborg²³, Carla P Rodriguez-Monserrate²⁴, Isabelle Vonder Muhll²⁵, Stephen Pylypchuk²⁵, Anthony Magalski²⁶, Frank Han²⁷, Adam M Lubert²⁸, Joseph Kay²⁹, Elizabeth Yeung²⁹, Jolien Roos-Hesselink⁵, David Baker³⁰, David S Celermajer³⁰, Luke J Burchill³¹, William M Wilson³¹, Joshua Wong³¹, Shelby Kutty³², Alexander R Opotowsky³³

Affiliations

¹ Knight Cardiovascular Institute, Oregon Health and Science University, Portland, Oregon, USA. Electronic address: brobergc@ohsu.edu.
² Knight Cardiovascular Institute, Oregon Health and Science University, Portland, Oregon, USA; Amsterdam University Medical Center, Amsterdam, the Netherlands.
³ School of Public Health, Oregon Health and Science University, Portland, Oregon, USA.
⁴ UCLA Medical Center, Los Angeles, California, USA.
⁵ Erasmus Medical Center, Rotterdam, the Netherlands.
⁶ Children's Hospital of Wisconsin, Milwaukee, Wisconsin, USA.
⁷ University of Washington Medical Center and Seattle Children's Hospital, Seattle, Washington, USA.
⁸ Emory University Hospital, Atlanta, Georgia, USA.
⁹ Ochsner Medical Center, New Orleans, Louisiana, USA.
¹⁰ Children's National Hospital, Washington, DC, USA.
¹¹ University of Michigan Medical Center, Ann Arbor, Michigan, USA.
¹² Krannert Institute of Cardiology, Indianapolis, Indiana, USA.
¹³ Knight Cardiovascular Institute, Oregon Health and Science University, Portland, Oregon, USA.
¹⁴ Providence Spokane, Spokane, Washington, USA.
¹⁵ Monaldi Hospital, Napoli, Italy.
¹⁶ University Hospital Motol, Prague, Czech Republic.
¹⁷ Stanford University, School of Medicine, Departments of Pediatrics and Medicine, Palo Alto, California, USA.
¹⁸ St. Paul's Hospital, Division of Cardiology, University of British Columbia, Vancouver, British Columbia, Canada.
¹⁹ Children's Hospital, Omaha & University of Nebraska Medical Center, Omaha, Nebraska, USA.
²⁰ Montreal Heart Institute, Montreal, Quebec, Canada.
²¹ Hospital Universitario Virgen Del Rocio, Sevilla, Spain.
²² Green Lane Paediatric and Congenital Cardiac Service, Auckland City Hospital, Auckland, New Zealand.
²³ Institute of Medicine, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden.
²⁴ Boston Children's Hospital, and Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, USA; Department of Medicine, Division of Cardiology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
²⁵ University of Alberta, Edmonton, Alberta, Canada.
²⁶ Saint Luke's Hospital, Kansas City, Missouri, USA.
²⁷ University of Mississippi Medical Center, Jackson, Mississippi, USA.
²⁸ Cincinnati Children's Hospital Medical Center, Heart Institute, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA.
²⁹ Colorado University School of Medicine, Denver, Colorado, USA.
³⁰ University of Sydney and Royal Prince Alfred Hospital, Sydney, New South Wales, Australia.
³¹ Royal Melbourne Hospital, Department of Medicine, Melbourne, Victoria, Australia.
³² Johns Hopkins University, Baltimore, Maryland, USA.
³³ Boston Children's Hospital, and Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, USA; Department of Medicine, Division of Cardiology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA; Cincinnati Children's Hospital Medical Center, Heart Institute, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA.

PMID: 36049802
DOI: 10.1016/j.jacc.2022.06.020

Abstract

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.

Keywords: QRS duration; atrial arrhythmia; congenital heart disease; congestive heart failure; systemic right ventricle; transposition of the great arteries.

Publication types

Multicenter Study
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't

MeSH terms

Adult
Arterial Switch Operation* / adverse effects
Arteries
Female
Follow-Up Studies
Heart Failure* / epidemiology
Heart Failure* / etiology
Humans
Male
Retrospective Studies
Transposition of Great Vessels* / surgery
Treatment Outcome

Grants and funding

UL1 TR002369/TR/NCATS NIH HHS/United States