BACKGROUND: Congenitally corrected transposition of the great arteries (ccTGA) is a rare clinical condition in which the morphologically right ventricle sustains systemic circulation. This congenital heart anomaly exposes patients with ccTGA to adverse events over time. Strategies to identify persons who are at high risk of clinical events will be crucial for improving patient outcomes. Thus the aim of this study was to identify screening tools that enable morbidity and mortality risk stratification in adults with ccTGA. METHODS: This was a prospective observational study. Electrocardiography, laboratory testing, echocardiography, and cardiopulmonary exercise testing were performed at baseline. A Cox proportional hazards regression analysis was conducted to establish determinants of composite clinical endpoints, including death, heart transplantation, systemic ventricular device assist implantation, worsening of heart failure, vascular events, tricuspid valve regurgitation requiring intervention, and clinically relevant arrhythmias. RESULTS: Fifty-one patients-30 male and 21 female-with a mean age of 36 years were studied. During a median follow-up period of 3.15 years, 19 patients experienced 39 clinical events. Detectable high-sensitivity troponin T (hsTnT) combined with echocardiography-derived systemic right ventricular end-diastolic (sRVED) area were the best predictors of adverse outcomes (hazard ratio [HR] = 6.25, P = 0.02 and HR = 1.05, P = 0.02, respectively). CONCLUSIONS: A combination of detectable hsTnT and an increased sRVED area are the best predictors of adverse clinical events in adults with ccTGA. This observation may be useful to guide follow-up, as both risk determinants are widely available and simple to obtain in everyday practice.
BACKGROUND: Congenitally corrected transposition of the great arteries (ccTGA) is a rare clinical condition in which the morphologically right ventricle sustains systemic circulation. This congenital heart anomaly exposes patients with ccTGA to adverse events over time. Strategies to identify persons who are at high risk of clinical events will be crucial for improving patient outcomes. Thus the aim of this study was to identify screening tools that enable morbidity and mortality risk stratification in adults with ccTGA. METHODS: This was a prospective observational study. Electrocardiography, laboratory testing, echocardiography, and cardiopulmonary exercise testing were performed at baseline. A Cox proportional hazards regression analysis was conducted to establish determinants of composite clinical endpoints, including death, heart transplantation, systemic ventricular device assist implantation, worsening of heart failure, vascular events, tricuspid valve regurgitation requiring intervention, and clinically relevant arrhythmias. RESULTS: Fifty-one patients-30 male and 21 female-with a mean age of 36 years were studied. During a median follow-up period of 3.15 years, 19 patients experienced 39 clinical events. Detectable high-sensitivity troponin T (hsTnT) combined with echocardiography-derived systemic right ventricular end-diastolic (sRVED) area were the best predictors of adverse outcomes (hazard ratio [HR] = 6.25, P = 0.02 and HR = 1.05, P = 0.02, respectively). CONCLUSIONS: A combination of detectable hsTnT and an increased sRVED area are the best predictors of adverse clinical events in adults with ccTGA. This observation may be useful to guide follow-up, as both risk determinants are widely available and simple to obtain in everyday practice.
Authors: Ewa Kowalik; Anna Kwiatek-Wrzosek; Anna Klisiewicz; Anna Lutynska; Elzbieta Katarzyna Biernacka; Miroslaw Kowalski; Piotr Hoffman Journal: Anatol J Cardiol Date: 2020-08 Impact factor: 1.596