End-stage heart failure in congenitally corrected transposition of the great arteries: a multicentre study

AC van Dissel, AR Opotowsky, LJ Burchill, J Aboulhosn, J Grewal, AM Lubert, P Antonova, S Shah, T Cotts, AS John, WA Kay, C DeZorzi, A Magalski, F Han, D Baker, J Kay, ELZB Yeung, I Vonder Muhll, S Pylypchuk, MC KuoJ Nicolarsen, B Sarubbi, F Fusco, SM Jameson, J Cramer, T Gupta, P Gallego, C O'Donnell, J Hannah, M Dellborg, RM Kauling, S Ginde, EV Krieger, F Rodriguez, P Dehghani, S Kutty, JS Wong, WM Wilson, CP Rodriguez-Monserrate, J Roos-Hesselink, DS Celermajer, P Khairy, CS Broberg*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

5 Citations (Scopus)
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BACKGROUND AND AIMS: For patients with congenitally corrected transposition of the great arteries (ccTGA), factors associated with progression to end-stage congestive heart failure (CHF) remain largely unclear. METHODS: This multicentre, retrospective cohort study included adults with ccTGA seen at a congenital heart disease centre. Clinical data from initial and most recent visits were obtained. The composite primary outcome was mechanical circulatory support, heart transplantation, or death. RESULTS: From 558 patients (48% female, age at first visit 36 ± 14.2 years, median follow-up 8.7 years), the event rate of the primary outcome was 15.4 per 1000 person-years (11 mechanical circulatory support implantations, 12 transplantations, and 52 deaths). Patients experiencing the primary outcome were older and more likely to have a history of atrial arrhythmia. The primary outcome was highest in those with both moderate/severe right ventricular (RV) dysfunction and tricuspid regurgitation (n = 110, 31 events) and uncommon in those with mild/less RV dysfunction and tricuspid regurgitation (n = 181, 13 events, P < .001). Outcomes were not different based on anatomic complexity and history of tricuspid valve surgery or of subpulmonic obstruction. New CHF admission or ventricular arrhythmia was associated with the primary outcome. Individuals who underwent childhood surgery had more adverse outcomes than age- and sex-matched controls. Multivariable Cox regression analysis identified older age, prior CHF admission, and severe RV dysfunction as independent predictors for the primary outcome. CONCLUSIONS: Patients with ccTGA have variable deterioration to end-stage heart failure or death over time, commonly between their fifth and sixth decades. Predictors include arrhythmic and CHF events and severe RV dysfunction but not anatomy or need for tricuspid valve surgery.

Original languageEnglish
Pages (from-to)3278-3291
Number of pages14
JournalEuropean Heart Journal
Issue number34
Publication statusPublished - 7 Sept 2023

Bibliographical note

Funding Information:
The study was funded by a joint grant from the Children’s Heart Foundation and the American Heart Association (17GRNT33670334). A.R.O. was also supported by the Heart Institute Research Core (HIRC) at Cincinnati Children’s Hospital and the Dunlevie Family Fund. REDCap is supported by the National Institutes of Health (UL1TR002369).

Publisher Copyright:
© 2023 The Author(s). Published by Oxford University Press on behalf of the European Society of Cardiology.


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