Korean Circ J.  2009 Aug;39(8):340-342. 10.4070/kcj.2009.39.8.340.

Non-Ischemic Cardiomyopathy Attributed to Adult Myotonic Dystrophy

• Park HK
• Park WH
• Song DG
• Kim TG
• Min BY
• Lee K
• Park JI

• KMID: 2225685
• DOI: http://doi.org/10.4070/kcj.2009.39.8.340

Abstract

In patients with myotonic dystrophy (MD), impairment of the conduction system is a common and progressive finding. However, only a few cases of MD with cardiomyopathy have been reported. Herein we report a case of MD with progressive non-ischemic cardiomyopathy and severe electrocardiographic abnormalities.

Keyword

Myotonic dystrophy; Cardiomyopathy

MeSH Terms

Adult
Cardiomyopathies
Electrocardiography
Humans
Myotonic Dystrophy

Figure

• 

  Fig. 1 A: Rectus femoralis muscle biopsy shows atrophy and degenerated fibers with internal nuclei (arrow). B: 12-lead EKG shows first-degree AV block (PR interval, 220 msec) and intraventricular conduction delay (QRS duration, 142 msec). C: cardiac MRI did not show an abnormal signal intensity of the myocardium suggestive of fat infiltration or fibrosis. D: intermittent left bundle branch block was observed on 24-hour Holter monitoring. EKG: electrocardiogram, AV: atrioventricular.

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