Abstract
BackgroundMyocardial fibrosis is a common pathophysiological process that is related to ventricular remodeling in congenital heart disease. However, the presence, characteristics, and clinical significance of myocardial fibrosis in Ebstein’s anomaly have not been fully investigated. This study aimed to evaluate myocardial fibrosis using cardiovascular magnetic resonance (CMR) late gadolinium enhancement (LGE) and T1 mapping techniques, and to explore the significance of myocardial fibrosis in adolescent and adult patients with Ebstein’s anomaly.MethodsForty-four consecutive patients with unrepaired Ebstein’s anomaly (34.0 ± 16.2 years; 18 males), and an equal number of age- and gender-matched controls, were included. A comprehensive CMR protocol consisted of cine, LGE, and T1 mapping by modified Look-Locker inversion recovery (MOLLI) sequences were performed. Ventricular functional parameters, native T1, extracellular volume (ECV), and LGE were analyzed. Associations between myocardial fibrosis and disease severity, ventricular function, and NYHA classification were analyzed.ResultsLGE was found in 10 (22.7%) patients. Typical LGE in Ebstein’s anomaly was located in the endocardium of the septum within the right ventricle (RV). The LV ECV of Ebstein’s anomaly were significantly higher than those of the controls (30.0 ± 3.8% vs. 25.3 ± 2.3%, P < 0.001). An increased ECV was found to be independent of the existence of LGE. Positive LGE or higher ECV (≥30%) was associated with larger fRV volume, aRV volume, increased disease severity, and worse NYHA functional class. In addition, ECV was significantly correlated with the LV ejection fraction (P < 0.001).ConclusionsBoth focal and diffuse myocardial fibrosis were observed in adolescent and adult patients with Ebstein’s anomaly. Increased diffuse fibrosis is associated with worse LV function, increased Ebstein’s severity, and worse clinical status.
Highlights
Myocardial fibrosis is a common pathophysiological process that is related to ventricular remodeling in congenital heart disease
Patients were excluded from the study because of: age < 10 years (n = 12), no contrast was used (n = 2), poor image quality caused by due to persistent atrial fibrillation or unable to breath hold (n = 10), and T1 mapping not performed (n = 10)
Our study indicated that further longitudinal observation is needed to clarify the impact of myocardial fibrosis and remodeling in Ebstein’s anomaly patients
Summary
Myocardial fibrosis is a common pathophysiological process that is related to ventricular remodeling in congenital heart disease. The presence, characteristics, and clinical significance of myocardial fibrosis in Ebstein’s anomaly have not been fully investigated. Ebstein’s anomaly is a rare congenital heart disease. It is considered a right ventricular (RV) myopathy with failure of delamination of the tricuspid leaflets. Compared with the tricuspid valve and RV abnormalities, the left ventricle (LV) of Ebstein’s anomaly is less studied. LV dysfunction was found in 7–50% patients with Ebstein’s anomaly and was associated with worse prognosis after surgery [7,8,9,10]. The mechanism of LV dysfunction in Ebstein’s anomaly is yet to be explored. Myocardial fibrosis was found in the left heart of decedents with Ebstein’s anomaly [11, 12]. Limited by the available techniques, to date, it has been difficult to explore the relationship between myocardial fibrosis and ventricular dysfunction in vivo
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