Clinical aspects of myocardial fibrosis in adults with Ebstein's anomaly.

Heart failure and arrhythmia are common complications in adults with Ebstein's anomaly. They may result not only from hemodynamic alterations, but also from myocardial fibrosis. Late gadolinium enhancement (LGE) by CMR enables the evaluation of myocardial fibrosis. The aim of the study was to asses the presence of LGE and its relation to clinical outcome. We studied a group of 37 unoperated adults aged 43.0 ± 14.4 years with Ebstein's anomaly from the congenital heart disease outpatient clinic. Study protocol included: cardiopulmonary test, assessment of supraventricular arrhythmia (SVA), and CMR with evaluation of cardiac chambers' morphology and function, and presence of LGE. Variables following normal distribution were shown as mean ± SD if otherwise median (range) was applied. Fibrosis was found in 18 patients (48.6%) and was distributed as follows: 12 patients (32.4%) in the right atrium, 12 (32.4%) in the atrialized right ventricle, and 2 (5.4%) in the functional right ventricle. In patients with fibrosis, the tricuspid regurgitation fraction was bigger (48.3 ± 19.7 vs. 36.1 ± 22.6%, p = 0.048) and SVA was more frequent [12 (66.7%) vs. 6 (31.6%), p = 0.046] when compared to patients without fibrosis. However, exercise capacity did not differ between patients with and without LGE (peak VO 24.0 ± 4.7 vs. 23.7 ± 4.4, p = 0.87). In adults with Ebstein's anomaly fibrosis estimated by LGE-CMR was localized in the right atrium and the right ventricle only. Volume overload resulting from tricuspid regurgitation might be a factor conducive to fibrosis. Myocardial fibrosis did not influence exercise capacity. Association between myocardial fibrosis and supraventricular arrhythmia was confirmed.