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. 2006 Aug;14(7-8):258-260, 261-262.

Pacing for conduction disturbances in Steinert's disease: a new indication for biventricular ICD?

SAM Said, JC Baart, WG de Voogt
PMCID: PMC2557190  PMID: 25696650

Abstract

Cardiac involvement in classical Steinert muscular dystrophy (dystrophia myotonica, MD1) is characterised by atrial arrhythmias, AV conduction disturbance, ventricular arrhythmias and heart failure. In MD1 patients complaints of fatigue and reduced exercise tolerance are well explained by the muscular weakness, but the same symptoms can be attributed to arrhythmia, atrioventricular block and heart failure. As cardiac pathology is often encountered in MD1 patients, an ECG, echocardiogram and Holter registrations should be performed on a routine basis.

We report on two patients with MD1 who developed Mobitz II block as initial presentation of cardiac disease.

Keywords: myotonic dystrophy, atrioventricular block pacing, atherosclerosis (coronary), heart failure (congestive), cardiomyopathy (dilated), sudden death

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Selected References

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

  1. Badano L., Autore C., Fragola P. V., Picelli A., Antonini G., Vichi R., Cannata D. Left ventricular myocardial function in myotonic dystrophy. Am J Cardiol. 1993 Apr 15;71(11):987–991. doi: 10.1016/0002-9149(93)90919-4. [DOI] [PubMed] [Google Scholar]
  2. Bécane H. M., Bonne G., Varnous S., Muchir A., Ortega V., Hammouda E. H., Urtizberea J. A., Lavergne T., Fardeau M., Eymard B. High incidence of sudden death with conduction system and myocardial disease due to lamins A and C gene mutation. Pacing Clin Electrophysiol. 2000 Nov;23(11 Pt 1):1661–1666. doi: 10.1046/j.1460-9592.2000.01661.x. [DOI] [PubMed] [Google Scholar]
  3. Forsberg H., Olofsson B. O., Eriksson A., Andersson S. Cardiac involvement in congenital myotonic dystrophy. Br Heart J. 1990 Feb;63(2):119–121. doi: 10.1136/hrt.63.2.119. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Fragola P. V., Autore C., Magni G., Antonini G., Picelli A., Cannata D. The natural course of cardiac conduction disturbances in myotonic dystrophy. Cardiology. 1991;79(2):93–98. doi: 10.1159/000174865. [DOI] [PubMed] [Google Scholar]
  5. 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]
  6. Hadian Djavid, Lowe Miriam R., Scott Luis R., Groh William J. Use of an insertable loop recorder in a myotonic dystrophy patient. J Cardiovasc Electrophysiol. 2002 Jan;13(1):72–73. doi: 10.1046/j.1540-8167.2002.00072.x. [DOI] [PubMed] [Google Scholar]
  7. Kuzmicic Calderón B., Valls Arara V., Brugada Terradellas J., Navarro Michel X., Navarro López F. Taquicardia ventricular monomórfica sostenida en la distrofia miotónica. Rev Esp Cardiol. 1994 Dec;47(12):843–846. [PubMed] [Google Scholar]
  8. Melacini P., Buja G., Fasoli G., Angelini C., Armani M., Scognamiglio R., Dalla Volta S. The natural history of cardiac involvement in myotonic dystrophy: an eight-year follow-up in 17 patients. Clin Cardiol. 1988 Apr;11(4):231–238. doi: 10.1002/clc.4960110407. [DOI] [PubMed] [Google Scholar]
  9. Ng W., Lau C. P. Cardiac arrhythmias as presenting symptoms in patients with limb-girdle muscular dystrophy. Int J Cardiol. 1997 Apr 18;59(2):157–160. doi: 10.1016/s0167-5273(96)02911-7. [DOI] [PubMed] [Google Scholar]
  10. Pelargonio G., Dello Russo A., Sanna T., De Martino G., Bellocci F. Myotonic dystrophy and the heart. Heart. 2002 Dec;88(6):665–670. doi: 10.1136/heart.88.6.665. [DOI] [PMC free article] [PubMed] [Google Scholar]
  11. Podczeck A., Graf M., Hief C., Kaltenbrunner W., Nürnberg M., Steinbach K. Elektrokardiographische und elektrophysiologische Befunde bei Patienten mit myotoner Muskeldystrophie. Z Kardiol. 1993 Aug;82(8):474–476. [PubMed] [Google Scholar]
  12. Rechavia E., Strasberg B., Agmon J. Unusual cardiac manifestations in a patient with myotonic muscular dystrophy. Int J Cardiol. 1986 Jun;11(3):349–352. doi: 10.1016/0167-5273(86)90039-2. [DOI] [PubMed] [Google Scholar]
  13. Tokgozoglu L. S., Ashizawa T., Pacifico A., Armstrong R. M., Epstein H. F., Zoghbi W. A. Cardiac involvement in a large kindred with myotonic dystrophy. Quantitative assessment and relation to size of CTG repeat expansion. JAMA. 1995 Sep 13;274(10):813–819. [PubMed] [Google Scholar]
  14. van Berlo Jop H., de Voogt Willem G., van der Kooi Anneke J., van Tintelen J. Peter, Bonne Gisèle, Yaou Rabah Ben, Duboc Denis, Rossenbacker Tom, Heidbüchel Hein, de Visser Marianne. Meta-analysis of clinical characteristics of 299 carriers of LMNA gene mutations: do lamin A/C mutations portend a high risk of sudden death? J Mol Med (Berl) 2004 Nov 13;83(1):79–83. doi: 10.1007/s00109-004-0589-1. [DOI] [PubMed] [Google Scholar]
  15. van der Kooi A. J., Ledderhof T. M., de Voogt W. G., Res C. J., Bouwsma G., Troost D., Busch H. F., Becker A. E., de Visser M. A newly recognized autosomal dominant limb girdle muscular dystrophy with cardiac involvement. Ann Neurol. 1996 May;39(5):636–642. doi: 10.1002/ana.410390513. [DOI] [PubMed] [Google Scholar]

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