Prognostic significance of right ventricular hypertrophy and systolic function in Anderson –Fabry disease

AbstractAimsRight ventricular hypertrophy (RVH) is a common finding in Anderson –Fabry disease (AFD), but the prognostic role of right ventricular (RV) involvement has never been assessed. The aim of our study was to evaluate the prognostic significance of RVH and RV systolic function in AFD.Methods and resultsForty ‐five AFD patients (56% male patients) with extensive baseline evaluation, including assessment of RVH and RV systolic function, were followed‐up for an average of 51.2 ± 11.4 months. RV systolic function was assessed by standard and tissue Doppler echocardiography. Cardiovascular events wer e defined as new‐onset atrial fibrillation (AF), sustained ventricular arrhythmias, heart failure, or pacemaker/implantable cardioverter defibrillator implantation; renal events were defined as progression to dialysis and/or renal transplantation or significant worsening of glomerular filtration r ate; and cerebrovascular events were defined as transient ischaemic attack or stroke.Fourteen patients (31.1%) presented RVH, while RV systolic function was normal in all cases. During the follow ‐up period, 13 patients (28.8%, 11 male) experienced 18 major events, including two deaths. Cardiovascular events occurred in eight patients (17.7%). The most common event was pacemaker/implantable cardioverter defibrillator implantation (six patients, 13.3%), followed by AF (three cases, 6.6%). O nly one case of worsening New York Heart Association class ...
Source: ESC Heart Failure - Category: Cardiology Authors: Tags: Original Research Article Source Type: research

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