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.
Facioscapulohumeral muscular dystrophy and occurrence of heart arrhythmia.
Trevisan CP, Pastorello E, Armani M, Angelini C, Nante G, Tomelleri G, Tonin P, Mongini T, Palmucci L, Galluzzi G, Tupler RG, Barchitta A.
Click here to read Abstract

Neuromuscul Disord. 2005 Jun;15(6):403-8. Epub 2005 Apr 21.
Subclinical cardiac involvement in patients with facioscapulohumeral muscular dystrophy.
Galetta F, Franzoni F, Sposito R, Plantinga Y, Femia FR, Galluzzi F, Rocchi A, Santoro G, Siciliano G.
Click here to read Abstract

Z Kardiol. 2005 May;94(5):348-54.
Facioscapulohumeral muscle dystrophy and heart disease  [Article in German]
Emmrich P, Ogunlade V, Gradistanac T, Daneschnejad S, Koch MC, Schober R.
Click here to read Abstract

Neurology. 1998 Nov;51(5):1454-6.
Cardiac involvement in genetically confirmed facioscapulohumeral muscular dystrophy.
Laforêt P, de Toma C, Eymard B, Becane HM, Jeanpierre M, Fardeau M, Duboc D.
Click here to read Abstract

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Health Information About Facioscapulohumeral Muscular Dystrophy (FSHD)

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The information on this website is provided for general informational and educational purposes only.  The FSH Society and this website do not provide medical advice or recommendations.  Licensed physicians and other medical professionals who are familiar with an individual’s specific health situation should be consulted for diagnosis and treatment of Facioscapulohumeral Muscular Dystrophy and any other medical conditions.  Neither the FSH Society nor any contributor to this website can be liable or responsible for any result derived from the use of this material.