Cardiac Involvement and FSHD
Cardiac complications in facioscapulohumeral muscular dystrophy (FSHD) are rare, and patients in general have normal longevity. This predisposes them to the usual age-related cardiac complications, and management of these problems is the same as in non-dystrophic patients.
The presence of cardiac abnormalities in FSHD is debated. While most authors have noted the presence of diverse electrocardiographic abnormalities, some have found no abnormalities on electrocardiography, chest radiography, Holter monitoring, or echocardiography. Nuclear scanning with thallium-201 has demonstrated diffuse defects consistent with diffuse fibrosis. 168 abnormalities in systolic time intervals on echocardiography 90 and elevations in atrial natriuretic peptide are consistent with subclinical cardiomyopathy.
Atrial paralysis, an unusual condition in which the atrium is devoid of electrical or mechanical activity, has been reported by several investigators. An in-depth investigation of cases of atrial paralysis found that 33% of all cases were associated with Emery-Dreifuss muscular dystrophy (EDMD). The literature states that the finding of atrial paralysis distinguishes EDMD from other similar dystrophies, such as FSHD.
Links to recent articles about cardiac involvement in FSHD
Eur Neurol. 2006;56(1):1-5. Epub 2006 Jun 27.
Neuromuscul Disord. 2005 Jun;15(6):403-8. Epub 2005 Apr 21.
Z Kardiol. 2005 May;94(5):348-54.
Neurology. 1998 Nov;51(5):1454-6.
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