Skip to main content
PMC home page
British Heart Journal logoLink to British Heart Journal
. 1988 Jan;59(1):47–52. doi: 10.1136/hrt.59.1.47

Electrocardiographic findings in myotonic dystrophy.

B O Olofsson 1, H Forsberg 1, S Andersson 1, P Bjerle 1, A Henriksson 1, I Wedin 1
PMCID: PMC1277071  PMID: 3342149

Abstract

Sixty five patients with myotonic dystrophy, from a defined population in northern Sweden with an extremely high prevalence of this disease, were examined by electrocardiography. The patients were subdivided into three groups according to the severity of the disease. Abnormal electrocardiograms were found in 6 (35%) patients with mild disease, 12 (50%) patients with moderate disease, and 23 (96%) patients with severe disease. First degree atrioventricular block and left anterior hemiblock were the most commonly encountered abnormalities in patients with mild and moderate disease, whereas atrial fibrillation and flutter, abnormal Q waves, and repolarisation abnormalities were more common in patients with severe disease. This study shows that the heart is often affected by myotonic dystrophy. These effects can be detected by electrocardiography in early and mild forms of the disease. The effect on the heart is progressive and clinically important atrial arrhythmias and electrocardiographic abnormalities which are useful in differential diagnosis are common in severe forms of the disease.

Full text

PDF
47

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Ahnve S. Correction of the QT interval for heart rate: review of different formulas and the use of Bazett's formula in myocardial infarction. Am Heart J. 1985 Mar;109(3 Pt 1):568–574. doi: 10.1016/0002-8703(85)90564-2. [DOI] [PubMed] [Google Scholar]
  2. Atarashi H., Saito H., Aoki H., Hayakawa H. A case of myotonic dystrophy associated with sick sinus syndrome. Jpn Circ J. 1981 Jul;45(7):763–768. doi: 10.1253/jcj.45.763. [DOI] [PubMed] [Google Scholar]
  3. Barrett P. A., Peter C. T., Swan H. J., Singh B. N., Mandel W. J. The frequency and prognostic significance of electrocardiographic abnormalities in clinically normal individuals. Prog Cardiovasc Dis. 1981 Jan-Feb;23(4):299–319. doi: 10.1016/0033-0620(81)90018-9. [DOI] [PubMed] [Google Scholar]
  4. Castellanos A., Jr, Lemberg L. Diagnosis of isolated and combined block in the bundle branches and the divisions of the left branch. Circulation. 1971 Jun;43(6):971–976. doi: 10.1161/01.cir.43.6.971. [DOI] [PubMed] [Google Scholar]
  5. Chagnon A., Vidal M. E. Maladie rythmique auriculaire au cours d'une myotonie de Steinert. A propos d'un cas avec enquête familiale. Sem Hop. 1983 Feb 24;59(8):556–558. [PubMed] [Google Scholar]
  6. Church S. C. The heart in myotonia atrophica. Arch Intern Med. 1967 Feb;119(2):176–181. [PubMed] [Google Scholar]
  7. Grigg L. E., Chan W., Mond H. G., Vohra J. K., Downey W. F. Ventricular tachycardia and sudden death in myotonic dystrophy: clinical, electrophysiologic and pathologic features. J Am Coll Cardiol. 1985 Jul;6(1):254–256. doi: 10.1016/s0735-1097(85)80286-2. [DOI] [PubMed] [Google Scholar]
  8. Griggs R. C., Davis R. J., Anderson D. C., Dove J. T. Cardiac conduction in myotonic dystrophy. Am J Med. 1975 Jul;59(1):37–42. doi: 10.1016/0002-9343(75)90319-8. [DOI] [PubMed] [Google Scholar]
  9. Harper P. S. The genetics of muscular dystrophies. Prog Med Genet. 1985;6:53–90. [PubMed] [Google Scholar]
  10. Hartwig G. B., Rao K. R., Radoff F. M., Coleman R. E., Jones R. H., Roses A. D. Radionuclide angiocardiographic analysis of myocardial function in myotonic muscular dystrophy. Neurology. 1983 May;33(5):657–660. doi: 10.1212/wnl.33.5.657. [DOI] [PubMed] [Google Scholar]
  11. Komajda M., Frank R., Vedel J., Fontaine G., Petitot J. C., Grosgogeat Y. Intracardiac conduction defects in dystrophia myotonica. Electrophysiological study of 12 cases. Br Heart J. 1980 Mar;43(3):315–320. doi: 10.1136/hrt.43.3.315. [DOI] [PMC free article] [PubMed] [Google Scholar]
  12. PAYNE C. A., GREENFIELD J. C., Jr Electrocardiographic abnormalities associated with myotonic dystrophy. Am Heart J. 1963 Apr;65:436–440. doi: 10.1016/0002-8703(63)90092-9. [DOI] [PubMed] [Google Scholar]
  13. PETKOVICH N. J., DUNN M., REED W. MYOTONIA DYSTROPHICA WITH A-V DISSOCIATION AND STOKES-ADAMS ATTACKS: A CASE REPORT AND REVIEW OF THE LITERATURE. Am Heart J. 1964 Sep;68:391–396. doi: 10.1016/0002-8703(64)90307-2. [DOI] [PubMed] [Google Scholar]
  14. Perloff J. K., Stevenson W. G., Roberts N. K., Cabeen W., Weiss J. Cardiac involvement in myotonic muscular dystrophy (Steinert's disease): a prospective study of 25 patients. Am J Cardiol. 1984 Nov 1;54(8):1074–1081. doi: 10.1016/s0002-9149(84)80147-2. [DOI] [PubMed] [Google Scholar]
  15. Prystowsky E. N., Pritchett E. L., Roses A. D., Gallagher J. The natural history of conduction system disease in myotonic muscular dystrophy as determined by serial electrophysiologic studies. Circulation. 1979 Dec;60(6):1360–1364. doi: 10.1161/01.cir.60.6.1360. [DOI] [PubMed] [Google Scholar]
  16. Rausing A. Focal myocarditis in familial dystrophia myotonica. Br Heart J. 1972 Dec;34(12):1292–1294. doi: 10.1136/hrt.34.12.1292. [DOI] [PMC free article] [PubMed] [Google Scholar]
  17. Reeves W. C., Griggs R., Nanda N. C., Thomson K., Gramiak R. Echocardiographic evaluation of cardiac abnormalities in Duchenne's dystrophy and myotonic muscular dystrophy. Arch Neurol. 1980 May;37(5):273–277. doi: 10.1001/archneur.1980.00500540051004. [DOI] [PubMed] [Google Scholar]
  18. Rüdel R., Lehmann-Horn F. Membrane changes in cells from myotonia patients. Physiol Rev. 1985 Apr;65(2):310–356. doi: 10.1152/physrev.1985.65.2.310. [DOI] [PubMed] [Google Scholar]
  19. Streib E. W., Meyers D. G., Sun S. F. Mitral valve prolapse in myotonic dystrophy. Muscle Nerve. 1985 Oct;8(8):650–653. doi: 10.1002/mus.880080804. [DOI] [PubMed] [Google Scholar]
  20. Uemura N., Tanaka H., Niimura T., Hashiguchi N., Yoshimura M., Terashi S., Kanehisa T. Electrophysiological and histological abnormalities of the heart in myotonic dystrophy. Am Heart J. 1973 Nov;86(5):616–624. doi: 10.1016/0002-8703(73)90338-4. [DOI] [PubMed] [Google Scholar]
  21. Viitasalo M. T., Kala R., Karli P., Eisalo A. Ambulatory electrocardiographic recording in mild or moderate myotonic dystrophy and myotonia congenita (Thomsen's disease). J Neurol Sci. 1983 Dec;62(1-3):181–190. doi: 10.1016/0022-510x(83)90198-3. [DOI] [PubMed] [Google Scholar]

Articles from British Heart Journal are provided here courtesy of BMJ Publishing Group

RESOURCES