Long-term outcome of patients with transposition of the great arteries and a systemic right ventricle: A systematic review and meta-analysis

BackgroundPatients with a transposition of the great arteries (TGA) and a systemic right ventricle are at risk of heart failure (HF) development, arrhythmia and early mortality. Prognostic evaluations in clinical studies are hampered by small sample sizes and single-centred approaches. We aimed to investigate yearly rate of outcome and factors affecting it.MethodsA systematic literature search of four electronic databases (PubMed, EMBASE, Web of Science and Scopus) was conducted from inception to June 2022. Studies reporting the association of a systemic right ventricle with mortality with a minimal follow-up of 2 years during adulthood were selected. Incidence of HF hospitalization and/or arrhythmia were captured as additional endpoints. For each outcome, a summary effect estimate was calculated.ResultsFrom a total of 3891 identified records, 56 studies met the selection criteria. These studies described the follow-up (on average 7.27 years) of 5358 systemic right ventricle patients. The mortality incidence was 1.3 (1–1.7) per 100 patients/year. The incidence of HF hospitalization was 2.6 (1.9–3.7) per 100 patients/year. Predictors of poor outcome were a lower left ventricular (LV) and right ventricular ejection fraction (RVEF) (standardized mean differences (SMD) of −0.43 (−0.77 to −0.09) and − 0.85 (−1.35 to −0.35), respectively), higher plasma concentrations of NT-proBNP (SMD of 1.24 (0.49–1.99)), and NYHA class ≥2 (risk ratio of 2.31 (1.57–3.39)).ConclusionsTGA patients with a systemic right ventricle have increased incidence of mortality and HF hospitalization. A lower LVEF and RVEF, higher levels of NT-proBNP and NYHA class ≥2 are associated with poor outcome.