Cardiac Abnormalities in Type 1 Facioscapulohumeral Muscular Dystrophy. Issue 4 (June 2017)

• Record Type: Journal Article
• Authors: Labombarda, Fabien; Maurice, Maxime; Simon, Jean-Philippe; Legallois, Damien; Guyant-Maréchal, Lucie; Bedat-Millet, Anne-Laure; Merle, Philippe; Saloux, Eric; Chapon, Françoise; Milliez, Paul
• Is Part Of: Journal of clinical neuromuscular disease. Volume 18:Issue 4(2017:Jun.)
• Publication Date: 2017-06
• Subjects: fascioscapulohumeral dystrophy -- heart; Neuromuscular diseases -- Periodicals; 616.744005
• DOI: 10.1097/CND.0000000000000144
• Languages: English
• ISSNs: 1522-0443

Abstract

Abstract: Objectives: We conducted a retrospective study to characterize the cardiac complications in patients with genetically confirmed type 1 facioscapulohumeral dystrophy. Methods: We reviewed baseline cardiac investigations, including electrocardiogram, Holter electrocardiogram and echocardiogram, as well as cardiac complications that occurred during follow-up in 56 adult patients (37 men, mean duration of disease: 20 years). Results: Baseline evaluation revealed minor cardiac anomalies in 23 patients including incomplete right bundle branch block (iRBBB) in 13 patients (23%). Over a mean follow-up period of 7.2 years, there was no cardiac death, no patient developed cardiomyopathy, and 28 patients (50%) experienced cardiac anomalies. Among these patients, 3 had one or more major events (heart failure and/or atrial fibrillation). The remaining 25 patients presented minor cardiac anomalies of which iRBBB was the most frequent (25%). Conclusions: Cardiac anomalies identified during the follow-up of patients with type 1 facioscapulohumeral dystrophy are mainly minor anomalies, dominated by the iRBBB.