Background and Purpose-Emery-Dreifuss muscular dystrophy (EDMD) is a rare inherited disorder associated with cardiac involvement. We investigated the spectrum and relevance of the cardiac manifestations of EDMD, focusing on bradyarrhythmias and tachyarrhythmias (including atrial fibrillation/flutter), embolic stroke, and heart failure. Methods and Results-Eighteen patients (age 42.8+/-19.6 years) with genetically confirmed X-linked (n=10, including 3 carriers) or autosomal dominant (n=8) EDMD were followed for a period ranging from 1 to 30 years in a research center for neuromuscular diseases and in a university cardiological department. Pacemakers were required by 10 of 18 (56%) patients for bradyarrhythmia, and related complications occurred in 3 of 10 (30%) cases. Atrial fibrillation/flutter developed in 11 of 18 (61%) patients, with atrial standstill subsequently occurring in 5 of 11 (45%) cases and embolic stroke (most often disabling) in 4 of 11 (36%). Heart failure requiring transplantation occurred in 1 of 18 (6%) patients, and asymptomatic left ventricular dysfunction in a further 3 (17%). No relationship was evident between neuromuscular impairment and cardiac involvement. Conclusions-Both X-linked and autosomal dominant EDMD patients risk not only bradyarrhythmia (requiring pacemaker implant) but also atrial fibrillation/flutter, which often anticipates atrial standstill and can cause disabling embolic stroke at a relatively young age. Antithromboembolic prophylaxis has to be recommended in EDMD patients with atrial fibrillation/flutter or atrial standstill. With careful monitoring, survival after pacemaker implant may be long. Heart failure, which seems to occur only in a minority of patients, may be severe.
Clinical relevance of atrial fibrillation/flutter, stroke, pacemaker implant, and heart failure in Emery-Dreifuss muscular dystrophy - A long-term longitudinal study / Boriani, Giuseppe; Gallina, M; Merlini, L; Bonne, G; Toniolo, D; Amati, S; Biffi, M; Martignani, C; Frabetti, L; Bonvicini, M; Rapezzi, C; Branzi, A.. - In: STROKE. - ISSN 0039-2499. - 34:4(2003), pp. 901-908.
Data di pubblicazione: | 2003 |
Titolo: | Clinical relevance of atrial fibrillation/flutter, stroke, pacemaker implant, and heart failure in Emery-Dreifuss muscular dystrophy - A long-term longitudinal study |
Autore/i: | Boriani, Giuseppe; Gallina, M; Merlini, L; Bonne, G; Toniolo, D; Amati, S; Biffi, M; Martignani, C; Frabetti, L; Bonvicini, M; Rapezzi, C; Branzi, A. |
Autore/i UNIMORE: | |
Digital Object Identifier (DOI): | http://dx.doi.org/10.1161/01.STR.0000064322.47667.49 |
Rivista: | |
Volume: | 34 |
Fascicolo: | 4 |
Pagina iniziale: | 901 |
Pagina finale: | 908 |
Codice identificativo ISI: | WOS:000182014300023 |
Codice identificativo Scopus: | 2-s2.0-0037383576 |
Codice identificativo Pubmed: | 12649505 |
Citazione: | Clinical relevance of atrial fibrillation/flutter, stroke, pacemaker implant, and heart failure in Emery-Dreifuss muscular dystrophy - A long-term longitudinal study / Boriani, Giuseppe; Gallina, M; Merlini, L; Bonne, G; Toniolo, D; Amati, S; Biffi, M; Martignani, C; Frabetti, L; Bonvicini, M; Rapezzi, C; Branzi, A.. - In: STROKE. - ISSN 0039-2499. - 34:4(2003), pp. 901-908. |
Tipologia | Articolo su rivista |
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