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.

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

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

Children's Hospital of Wisconsin Milwaukee Wisconsin USA

Children's Hospital Omaha and University of Nebraska Medical Center Omaha Nebraska USA

Children's National Hospital Washington DC USA

Cincinnati Children's Hospital Medical Center Heart Institute Department of Pediatrics University of Cincinnati College of Medicine Cincinnati Ohio USA

Colorado University School of Medicine Denver Colorado USA

Emory University Hospital Atlanta Georgia USA

Erasmus Medical Center Rotterdam the Netherlands

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

Hospital Universitario Virgen Del Rocio Sevilla Spain

Institute of Medicine Sahlgrenska Academy University of Gothenburg Gothenburg Sweden

Johns Hopkins University Baltimore Maryland USA

Knight Cardiovascular Institute Oregon Health and Science University Portland Oregon USA

Knight Cardiovascular Institute Oregon Health and Science University Portland Oregon USA; Amsterdam University Medical Center Amsterdam the Netherlands

Krannert Institute of Cardiology Indianapolis Indiana USA

Monaldi Hospital Napoli Italy

Montreal Heart Institute Montreal Quebec Canada

Ochsner Medical Center New Orleans Louisiana USA

Providence Spokane Spokane Washington USA

Royal Melbourne Hospital Department of Medicine Melbourne Victoria Australia

Saint Luke's Hospital Kansas City Missouri USA

School of Public Health Oregon Health and Science University Portland Oregon USA

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

Stanford University School of Medicine Departments of Pediatrics and Medicine Palo Alto California USA

UCLA Medical Center Los Angeles California USA

University Hospital Motol Prague Czech Republic

University of Alberta Edmonton Alberta Canada

University of Michigan Medical Center Ann Arbor Michigan USA

University of Mississippi Medical Center Jackson Mississippi USA

University of Sydney and Royal Prince Alfred Hospital Sydney New South Wales Australia

University of Washington Medical Center and Seattle Children's Hospital Seattle Washington USA

J Am Coll Cardiol. 2022 Sep 6;80(10):964-966. doi: 10.1016/j.jacc.2022.06.021. PubMed

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