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.

Children's Hospital of Wisconsin Milwaukee WI USA

Children's Hospital University of Nebraska Medical Centre Omaha NE USA

Children's National Hospital Washington DC USA

Colorado University School of Medicine Denver CO USA

Department of Clinical and Experimental Cardiology Amsterdam UMC University of Amsterdam Amsterdam The Netherlands

Department of Medicine Royal Melbourne Hospital Melbourne Australia

Department of Paediatrics Cincinnati Children's Hospital Medical Centre Heart Institute University of Cincinnati College of Medicine Cincinnati OH USA

Departments of Paediatrics and Cardiovascular Medicine School of Medicine Stanford University Palo Alto CA USA

Division of Cardiology St Paul's Hospital University of British Columbia Vancouver BC Canada

Emory University School of Medicine Atlanta GA USA

Erasmus Medical Centre Rotterdam The Netherlands

European Reference Network for Rare Low Prevalence and Complex Disease of the Heart ERN GUARD Heart

Green Lane Paediatric and Congenital Cardiac Service Auckland City Hospital Auckland New Zealand

Harvard Medical School Boston Children's Hospital Boston MA USA

Hospital Universitario Virgen Del Rocio Sevilla Spain

Institute of Medicine Sahlgrenska Academy University of Gothenburg Gothenburg Sweden

Johns Hopkins University Baltimore MD USA

Knight Cardiovascular Institute Oregon Health and Science University 3181 SW Sam Jackson Pk Rd Portland OR 97221 USA

Mercy Heart Institute Cincinnati OH USA

Monaldi Hospital Napoli Italy

Montreal Heart Institute Université de Montréal Montreal Canada

Ochsner Medical Centre New Orleans LA USA

Providence Spokane Spokane WA USA

Regina General Hospital Regina Canada

Ronald Regan UCLA Medical Centre Los Angeles CA USA

The University of Sydney and Royal Prince Alfred Hospital Sydney Australia

University Hospital Motol Prague Czech Republic

University of Alberta Edmonton AB Canada

University of Illinois Chicago IL USA

University of Michigan Medical Centre Ann Arbor MI USA

University of Missouri Kansas City and Saint Luke's Hospital Kansas City MO USA

University of Washington Medical Centre and Seattle Children's Hospital Seattle WA USA

Eur Heart J. 2023 Sep 7;44(34):3292-3294. doi: 10.1093/eurheartj/ehad482. PubMed

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Propensity Score Analysis of Possible Medication Effects on Outcomes in Patients With Systemic Right Ventricles

Prognostic Significance of Hemodynamics in Patients With Transposition of the Great Arteries and Systemic Right Ventricle