Skip to main content
NCBI home page
British Heart Journal logoLink to British Heart Journal
. 1980 Mar;43(3):315–320. doi: 10.1136/hrt.43.3.315

Intracardiac conduction defects in dystrophia myotonica. Electrophysiological study of 12 cases.

M Komajda, R Frank, J Vedel, G Fontaine, J C Petitot, Y Grosgogeat
PMCID: PMC482281  PMID: 7437178

Abstract

Twelve patients with dystrophia myotonica had cardiac electrphysiological study for conduction disturbances (five cases) or for syncope (seven cases). Conduction disturbances were found in each case, being intranodal in two cases, intra-Hisian in three cases, or diffuse in seven cases. These findings are in agreement with those previously reported, and may be related to the high incidence of sudden death in these patients. Pacemakers are advocated in symptomatic patients, and in some asymptomatic patients with severe and diffuse lesions.

Full text

PDF
315

Selected References

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

  1. Church S. C. The heart in myotonia atrophica. Arch Intern Med. 1967 Feb;119(2):176–181. [PubMed] [Google Scholar]
  2. Coutte R., Dragodanne C., Frank R., Vachon J. M., Vedel J., Fontaine G., Grosgogeat Y. Périodes réfractaires de conduction intra-cardiaque. Définitions et valeurs normales chez l'homme. Coeur Med Interne. 1975 Oct-Dec;14(4):513–521. [PubMed] [Google Scholar]
  3. DeWIND L. T., JONES R. J. Cardiovascular observations in dystrophia myotonica. J Am Med Assoc. 1950 Sep 23;144(4):299–303. doi: 10.1001/jama.1950.02920040013004. [DOI] [PubMed] [Google Scholar]
  4. FEARRINGTON E. L., GIBSON T. C., CHURCHILL R. E. VECTORCARDIOGRAPHIC AND ELECTROCARDIOGRAPHIC FINDINGS IN MYOTONIA ATROPHICA. A STUDY EMPLOYING THE FRANK LEAD SYSTEM. Am Heart J. 1964 May;67:599–609. doi: 10.1016/0002-8703(64)90330-8. [DOI] [PubMed] [Google Scholar]
  5. Fontaine G., Gallais-Hamono F., Frank R., Grosgogeat Y. Méthode d'enregistrement du faisceau de his par voie externe. Nouv Presse Med. 1977 Nov 5;6(37):3433–3433. [PubMed] [Google Scholar]
  6. 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]
  7. Guérot C., Coste A., Valère P. E., Tricot R. L'épreuve à l'Ajmaline dans le diagnostic du bloc auriculo-ventriculaire paroxystique. Arch Mal Coeur Vaiss. 1973 Oct;66(10):1241–1253. [PubMed] [Google Scholar]
  8. Josephson M. E., Caracta A. R., Gallagher J. J., Damato A. N. Site of conduction disturbances in a family with myotonic dystrophy. Am J Cardiol. 1973 Jul;32(1):114–118. doi: 10.1016/s0002-9149(73)80096-7. [DOI] [PubMed] [Google Scholar]
  9. LITCHFIELD J. A. A-V dissociation in dystrophia myotonica. Br Heart J. 1953 Jul;15(3):357–359. doi: 10.1136/hrt.15.3.357. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Meilhac B., Fleury G., Caspi G., d'Arcy F. F., Heulin A., Di Matteo J. Troubles de la conduction sino-auriculaire et auriculo-ventriculaire dans la maladie de Steinert. A propos d'un cas. Arch Mal Coeur Vaiss. 1976 May;69(5):547–552. [PubMed] [Google Scholar]
  11. Montoyo J. V., Escribano J. C., Llamas A., Permanyer C., Soler Amigo J. Estudio de la conducción cardiaca en la enfermedad de Steinert. Rev Esp Cardiol. 1976 Sep-Oct;29(5):395–402. [PubMed] [Google Scholar]
  12. Narula O. S., Samet P., Javier R. P. Significance of the sinus-node recovery time. Circulation. 1972 Jan;45(1):140–158. doi: 10.1161/01.cir.45.1.140. [DOI] [PubMed] [Google Scholar]
  13. ORNDAHL G., THULESIUS O., ENESTROEM S., DEHLIN O. THE HEART IN MYOTONIC DISEASE. Acta Med Scand. 1964 Oct;176:479–491. doi: 10.1111/j.0954-6820.1964.tb00953.x. [DOI] [PubMed] [Google Scholar]
  14. 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]
  15. Roses A. D., Appel S. H. Muscle membrane protein kinase in myotonic muscular dystrophy. Nature. 1974 Jul 19;250(463):245–247. doi: 10.1038/250245a0. [DOI] [PubMed] [Google Scholar]
  16. SPILLANE J. D. The heart in myotonia atrophica. Br Heart J. 1951 Jul;13(3):343–347. doi: 10.1136/hrt.13.3.343. [DOI] [PMC free article] [PubMed] [Google Scholar]
  17. Scherlag B. J., Lau S. H., Helfant R. H., Berkowitz W. D., Stein E., Damato A. N. Catheter technique for recording His bundle activity in man. Circulation. 1969 Jan;39(1):13–18. doi: 10.1161/01.cir.39.1.13. [DOI] [PubMed] [Google Scholar]
  18. Schmitt J., Schmidt C., Clerget, Luporsi D. Les troubles cardiaques de la maldie de Steinert. Exploration endocavitaire de l'activité hisienne dans une série de 8 cas. Ann Med Interne (Paris) 1974 Feb;125(2):195–199. [PubMed] [Google Scholar]
  19. Strauss H. C., Saroff A. L., Bigger J. T., Jr, Giardina E. G. Premature atrial stimulation as a key to the understanding of sinoatrial conduction in man. Presentation of data and critical review of the literature. Circulation. 1973 Jan;47(1):86–93. doi: 10.1161/01.cir.47.1.86. [DOI] [PubMed] [Google Scholar]
  20. Thomson A. M. Dystrophia cordis myotonica studied by serial histology of the pacemaker and conducting system. J Pathol Bacteriol. 1968 Oct;96(2):285–295. doi: 10.1002/path.1700960205. [DOI] [PubMed] [Google Scholar]
  21. Théry C., Ketelers J. Y., Gosselin B., Likiefre J., Bethouart M., Warembourg H. Le bloc auriculo-ventriculaire de la maladie de Steinert. Etude électrophysiologique et histologique des voies de conduction. Arch Mal Coeur Vaiss. 1975 Oct;68(10):1087–1093. [PubMed] [Google Scholar]
  22. WELSH J. D., HAASE G. R., BYNUM T. E. MYOTONIC MUSCULAR DYSTROPHY; SYSTEMIC MANIFESTATIONS. Arch Intern Med. 1964 Nov;114:669–679. doi: 10.1001/archinte.1964.03860110139016. [DOI] [PubMed] [Google Scholar]
  23. Wit A. L., Damato A. N., Weiss M. B., Steiner C. Phenomenon of the gap in atrioventricular conduction in the human heart. Circ Res. 1970 Nov;27(5):679–689. doi: 10.1161/01.res.27.5.679. [DOI] [PubMed] [Google Scholar]

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

RESOURCES