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Heart. 1998 Jun;79(6):619-21.

Cardiac involvement in proximal myotonic myopathy.

Author information

1
Department of Cardiology, Georg-August-University, Göttingen, Germany.

Abstract

Proximal myotonic myopathy (PROMM) is a recently described autosomal dominantly inherited disorder resulting in proximal muscles weakness, myotonia, and cataracts. A few patients with cardiac involvement (sinus bradycardia, supraventricular bigeminy, conduction abnormalities) have been reported. The cases of three relatives with PROMM (weakness of neck flexors and proximal extremity muscles, calf hypertrophy, myotonia, cataracts) are reported: a 54 year old man, his 73 year old mother, and 66 year old aunt. All three presented with conduction abnormalities and one had repeated, life threatening, sustained monomorphic ventricular tachycardia. This illustrates that severe cardiac involvement may occur in PROMM.

PMID:
10078095
PMCID:
PMC1728733
DOI:
10.1136/hrt.79.6.619
[Indexed for MEDLINE]
Free PMC Article

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