BACKGROUND: Patients with transposition of the great arteries (TGA) and systemic right ventricle often confront significant adverse cardiac events. The prognostic significance of invasive hemodynamic parameters in this context remains uncertain. Our hypothesis is that the aortic pulsatility index and hemodynamic profiling utilizing invasive measures provide prognostic insights for patients with TGA and a systemic right ventricle. METHODS: This retrospective multicenter cohort study encompasses adults with TGA and a systemic right ventricle who underwent cardiac catheterization. Data collection, spanning from 1994 to 2020, encompasses clinical and hemodynamic parameters, including measured and calculated values such as pulmonary capillary wedge pressure, aortic pulsatility index, and cardiac index. Pulmonary capillary wedge pressure and cardiac index values were used to establish 4 distinct hemodynamic profiles. A pulmonary capillary wedge pressure of ≥15 mm Hg indicated congestion, termed wet, while a cardiac index <2.2 L/min per m2 signified inadequate perfusion, labeled cold. The primary outcome comprised a composite of all-cause death, heart transplantation, or the requirement for mechanical circulatory support. RESULTS: Of 1721 patients with TGA, 242 individuals with available invasive hemodynamic data were included. The median follow-up duration after cardiac catheterization was 11.4 (interquartile range, 7.5-15.9) years, with a mean age of 38.5±10.8 years at the time of cardiac catheterization. Among hemodynamic parameters, an aortic pulsatility index <1.5 emerged as a robust predictor of the primary outcome, with adjusted hazard ratios of 5.90 (95% CI, 3.01-11.62; P<0.001). Among the identified 4 hemodynamic profiles, the cold/wet profile was associated with the highest risk for the primary outcome, with an adjusted hazard ratio of 3.83 (95% CI, 1.63-9.02; P<0.001). CONCLUSIONS: A low aortic pulsatility index (<1.5) and the cold/wet hemodynamic profile are linked with an elevated risk of adverse long-term cardiac outcomes in patients with TGA and systemic right ventricle.

Amsterdam University Medical Center the Netherlands

Children's Hospital of Wisconsin Milwaukee

Children's Hospital Omaha and University of Nebraska Medical Center

Children's National Hospital Washington DC

Colorado University School of Medicine Denver

Department of Medicine Royal Melbourne Hospital Melbourne VIC Australia

Department of Pediatrics Boston Children's Hospital and Division of Cardiology Department of Medicine Brigham and Women's Hospital Harvard Medical School Boston MA

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

Departments of Pediatrics and Medicine School of Medicine Stanford University Palo Alto CA

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

Emory University Hospital Atlanta GA

Erasmus Medical Center Rotterdam the Netherlands

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

Hospital Universitario Virgen Del Rocio Sevilla Spain

Institute of Medicine Sahlgrenska Academy University of Gothenburg Sweden

Johns Hopkins University Baltimore MD

Knight Cardiovascular Institute Oregon Health and Science University Portland

Monaldi Hospital Napoli Italy

Montreal Heart Institute QC Canada

Ochsner Medical Center New Orleans LA

Providence Spokane WA

Regina General Hospital SK Canada

University Hospital Motol Prague Czech Republic

University of Alberta Edmonton AB Canada

University of California Los Angeles Medical Center

University of Chicago IL

University of Michigan Medical Center Ann Arbor

University of Missouri Kansas City and Saint Luke's Hospital

University of Sydney and Royal Prince Alfred Hospital NSW Australia

University of Washington Medical Center and Seattle Children's Hospital

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