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Cardiac Abnormalities in Type 1 Facioscapulohumeral Muscular Dystrophy

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.
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Contributeur : Eve Sorel <>
Soumis le : jeudi 3 octobre 2019 - 15:09:02
Dernière modification le : mercredi 4 décembre 2019 - 09:52:03



Fabien Labombarda, Maxime Maurice, Jean-Philippe Simon, Damien Legallois, Lucie Guyant-Maréchal, et al.. Cardiac Abnormalities in Type 1 Facioscapulohumeral Muscular Dystrophy. Journal of Clinical Neuromuscular Disease, Lippincott, Williams & Wilkins, 2017, 18 (4), pp.199-206. ⟨10.1097/CND.0000000000000144⟩. ⟨hal-02304808⟩



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