Myocardial extracellular volume is a non-invasive tissue marker of heart failure in patients with transposition of the great arteries and systemic right ventricle
- Resource Type
- article
- Authors
- Nadya Al-Wakeel-Marquard; Tiago Ferreira da Silva; Felix Berger; Titus Kuehne; Daniel R. Messroghli
- Source
- Frontiers in Pediatrics, Vol 10 (2022)
- Subject
- transposition of the great arteries
systemic right ventricle
cardiovascular magnetic resonance
extracellular volume
myocardial fibrosis
heart failure
Pediatrics
RJ1-570
- Language
- English
- ISSN
- 2296-2360
BackgroundFocal myocardial fibrosis in the systemic right ventricle (RV) is related to ventricular dysfunction and adverse outcome in patients with d-transposition of the great arteries (dTGA) post atrial redirection and those with congenitally corrected TGA (ccTGA). The role of diffuse fibrotic lesions in these conditions remains poorly understood. Our study aimed to investigate diffuse myocardial fibrosis by measuring extracellular volume (ECV) with cardiovascular magnetic resonance (CMR) and to explore correlations between ECV and clinical as well as functional markers of heart failure in patients with TGA and systemic RV.MethodsWe prospectively included dTGA and ccTGA patients aged ≥14 years and compared them to healthy controls. Standardized CMR included modified Look-Locker Inversion recovery T1 mapping to quantify diffuse myocardial fibrosis in the systemic RV and the subpulmonary left ventricle (LV). The centerline of RV and LV myocardium was marked with a line of interest tool to determine native and post-contrast T1 for quantification of ECV.ResultsIn total, 13 patients (dTGA: n = 8, ccTGA: n = 5) with a median age of 30.3 years were enrolled. LV ECV was higher in patients than in controls [34% (30%–41%) vs. 26% (23%–27%), p