van Dijk, Gaby Pons and van der Kooi, Elly and Behin, Anthony and Smeets, Joep and Timmermans, Janneke and van der Maarel, Silvère and Padberg, George and Voermans, Nicol and van Engelen, Baziel (2014) High prevalence of incomplete right bundle branch block in facioscapulohumeral muscular dystrophy without cardiac symptoms. Functional Neurology, 29 (3). pp. 159-165. ISSN 1971-3274
|
Text
article.pdf - Published Version Download (643kB) | Preview |
Abstract
The exact prevalence and nature of cardiac involvement in facioscapulohumeral muscular dystrophy (FSHD) is unknown. Nevertheless, the current opinion is that symptomatic cardiac disease is rare. We performed a cardiac screening [electrocardiogram (ECG) and echocardiography in the event of ECG abnormalities] in 75 genetically confirmed, ambulant FSHD patients without cardiac symptoms, with an eight-year follow-up of 57 patients, and compared the findings with results of previously performed cardiac screenings in the normal population. Baseline ECG demonstrated incomplete right bundle branch block (RBBB) in 33%, complete RBBB in 4%, and other minor abnormalities in 16%. Echocardiography showed no abnormalities. No significant changes were found after eight years of follow-up. Comparison with ECG abnormalities in the normal population showed a higher prevalence of incomplete RBBB (9.7 times higher) and of complete RBBB (4.8 times higher) in FSHD patients. This study in cardiac asymptomatic FSHD patients shows i) increased prevalence of incomplete RBBB in the absence of cardiomyopathy; ii) no progression of these abnormalities during eight years of follow-up. We conclude that FSHD patients without cardiac complaints do not need specific cardiac screening or surveillance. Furthermore, the increased prevalence of incomplete RBBB in the absence of cardiomyopathy suggests a selective involvement of the His-Purkinje system in FSHD.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | bundle branch block (BBB), facioscapulohumeral muscular dystrophy (FSHD), electrocardiography (ECG) |
| Subjects: | 600 Tecnologia - Scienze applicate > 610 Medicina e salute (Classificare qui la tecnologia dei servizi medici) |
| Depositing User: | Marina Spanti |
| Date Deposited: | 15 Dec 2015 15:51 |
| Last Modified: | 15 Dec 2015 15:51 |
| URI: | http://eprints.bice.rm.cnr.it/id/eprint/11158 |
Actions (login required)
 |
View Item |
