The natural history of cardiac involvement in myotonic dystrophy: An eight-year follow-up in 17 patients

P. Melacini, G. Buja, G. Fasoli, C. Angelini, M. Armani, R. Scognamiglio, S. D. Volta

Research output: Contribution to journalArticle

Abstract

We evaluated the progression of conduction system and myocardial disease in 17 asymptomatic myotonic dystrophy patients by clinical evaluation, electrocardiography, vector cardiography, and echocardiography. An exercise test was done in 10 patients. After 8 years, a follow-up study of 12 of the 17 original patients was done with a similar protocol. During this period, 2 patients died: one of sudded death while the other had acute left ventricular failure. In our first control study, we found EKG abnormalities in 15 of our patients, consisting mostly of conduction defects or pseudonecrotic patterns. In our second control, all patients had conduction system disease and, in addition, 3 of them had premature ventricular beats. One patient developed dilated cardiomyopathy. In 6 patients, structural involvement of the right ventricle was found. We conclude that even in asymptomatic myotonic dystrophy patients a conduction system deficit is present and progresses, and cardiac death may occur in about 12% of these patients.

Original languageEnglish
Pages (from-to)231-238
Number of pages8
JournalClinical Cardiology
Volume11
Issue number4
Publication statusPublished - 1988

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

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    Melacini, P., Buja, G., Fasoli, G., Angelini, C., Armani, M., Scognamiglio, R., & Volta, S. D. (1988). The natural history of cardiac involvement in myotonic dystrophy: An eight-year follow-up in 17 patients. Clinical Cardiology, 11(4), 231-238.