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.
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.
Authors: Allison Ducharme-Smith; Stefan Nicolau; C Anwar A Chahal; Kirstie Ducharme-Smith; Shujah Rehman; Keerthi Jaliparthy; Nadeem Khan; Christopher G Scott; Erik K St Louis; Teerin Liewluck; Virend K Somers; Grace Lin; Peter A Brady; Margherita Milone Journal: Front Neurol Date: 2021-05-24 Impact factor: 4.003
Authors: Johannes Schmid; Meinrad Beer; Andrea Berghold; Tatjana Stojakovic; Hubert Scharnagl; Benjamin Dieplinger; Stefan Quasthoff; Josepha S Binder; Peter P Rainer Journal: ESC Heart Fail Date: 2020-05-31
Authors: Elisabeta Bădilă; Iulia Ioana Lungu; Alexandru Mihai Grumezescu; Alexandru Scafa Udriște Journal: Medicina (Kaunas) Date: 2021-05-12 Impact factor: 2.430