Citation

Auer J, Pujol C, Maurer SJ, Nagdyman N, Ewert P, Tutarel O. J. Cardiovasc. Dev. Dis. 2021; 8(9).

Copyright

(Copyright © 2021, MDPI: Multidisciplinary Digital Publications Institute)

DOI

10.3390/jcdd8090113

PMID

unavailable

Abstract

BACKGROUND: Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital heart defect (CHD). Contemporary data regarding its outcome in adults are scarce.

METHODS: Retrospective, single-center study of all ccTGA patients over the age of 16 years treated at our center during the time period 2006-2018. Only patients with a biventricular circulation were included. The primary endpoint was all-cause mortality.

RESULTS: Altogether, 96 patients (mean age 32.8 ± 16.0 years, female 50%) with ccTGA and a systemic right ventricle (SRV) were included in the study. An additional CHD was present in 81 patients (84.4%); most common were a ventricular septal defect (VSD) and a left ventricular outflow tract obstruction. Out of the whole cohort, 45 (46.9%) had already undergone cardiac surgery at baseline. During a median follow-up of 6.5 (IQR 2.8-12.7) years, the primary endpoint occurred in 10 patients (10.8%). Cause of death was cardiac in nine patients and suicide in one. Hospitalizations due to heart failure occurred in 48 patients (51.6%). Upon univariate Cox analysis, an NYHA class ≥III, severe tricuspid regurgitation, severe SRV systolic impairment, as well as a reduced left ventricular systolic function were predictors of the primary endpoint. Upon multivariable analysis, only NYHA class ≥ III (HR: 18.66, CI 95%: 3.01-115.80, p = 0.0017) and a reduced left ventricular systolic function (HR: 7.36, CI 95%: 1.18-45.99, p = 0.038) remained as independent predictors.

CONCLUSIONS: Adults with ccTGA and an SRV are burdened with significant morbidity and mortality. Predictors for mortality are NYHA class and subpulmonary left ventricular function. © 2021 by the authors. Licensee MDPI, Basel, Switzerland.

Language: en

Keywords

adult; human; suicide; Mortality; female; male; exercise; cause of death; heart failure; hospitalization; disease severity; major clinical study; retrospective study; follow up; cohort analysis; heart transplantation; heart surgery; heart ventricle tachycardia; Article; heart ventricle septum defect; complete heart block; tricuspid valve regurgitation; all cause mortality; New York Heart Association class; Adult congenital heart disease; congenitally corrected transposition of the great arteries; heart atrium arrhythmia; heart left ventricle outflow tract obstruction; Transposition of the great arteries