This Article Figures Only Full Text Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Lazarus, A. Articles by Duboc, D. Search for Related Content PubMed PubMed Citation Articles by Lazarus, A. Articles by Duboc, D.

CLINICAL STUDY: ARRHYTHMIAS

Long-term follow-up of arrhythmias in patients with myotonic dystrophy treated by pacing

A multicenter diagnostic pacemaker study

Arnaud Lazarus, MD^*,*, Jean Varin, MD^*, Dominique Babuty, MD, Frédéric Anselme, MD, Joel Coste, MD^* and Denis Duboc, MD^*

^* Service de Cardiologie et de Biostatistique, Hôpital Cochin, Paris France
InParys, Saint Cloud, France
Service de Cardiologie, Hôpital Trousseau, Tours, France
Service de Cardiologie, Hôpital Charles Nicolle, Rouen, France

Manuscript received May 2, 2002; revised manuscript received June 3, 2002, accepted June 24, 2002.

^* Reprint requests and correspondence: Dr. Arnaud Lazarus, InParys, 12 rue Pasteur, 92210 Saint Cloud, France.
lazarus{at}inparys.com

OBJECTIVES: We hypothesized that pacemaker (PM) implantation in patients with myotonic dystrophy (MD) with a prolonged HV interval, even asymptomatic, may protect them against sudden death related to atrioventricular (AV) block. We sought to prospectively document the true incidence of AV block episodes in this high-risk population and accurately trace, in the long term, by the PM, the occurrence of arrhythmias that may remain undetected during conventional follow-up.

BACKGROUND: Myotonic dystrophy is associated with a high risk of sudden death, commonly attributed to AV block or ventricular arrhythmias, but cardiac pacing is only recommended as a secondary prevention.

METHODS: Patients with MD with an HV interval 70 ms, even in the absence of related symptoms, prospectively received a cardiac PM, including an algorithm capable of diagnosing episodes of bradycardia and tachyarrhythmias.

RESULTS: The population consisted of 49 patients (45.5 ± 8.9 years old) followed for 53.5 ± 27.2 months. Paroxysmal arrhythmias were recorded in 41 patients (83.7%), consisting of complete AV block (n = 21), sino-atrial block (n = 4), or atrial (n = 25) or ventricular (n = 13) tachyarrhythmias. No patient died of AV block during follow-up, but 10 deaths occurred, 4 of them sudden. An arrhythmic cause could be excluded by postmortem PM interrogation in two cases of typical sudden death.

CONCLUSIONS: Arrhythmias are common in patients with MD with infrahisian conduction abnormalities. The prophylactic implantation of a pacing system when the HV interval is 70 ms seems appropriate. The PM protects the patient against the clinical consequences of paroxysmal profound bradycardia and facilitates the diagnosis and management of frequent paroxysmal tachyarrhythmias.

Abbreviations and Acronyms   AV   atrioventricular   ECG   electrocardiogram/electrocardiographic   EP   electrophysiologic   MD   myotonic dystrophy   PM   pacemaker   SA   sino-atrial

This article has been cited by other articles:

				V. A. Sansone, G. De Ambroggi, A. Zanolini, M. Panzeri, F. Sardanelli, R. Cappato, G. Meola, and L. De Ambroggi Long-term follow-up free of ventricular fibrillation recurrence after resuscitated cardiac arrest in a myotonic dystrophy type 1 patient Europace, September 1, 2009; 11(9): 1243 - 1244. [Abstract] [Full Text] [PDF]

				A. D. Russo, M. Pace, F. Bellocci, M. C.E. Hermans, C. G. Faber, Y. M. Pinto, B. Vrtovec, F. Haddad, A. A. Sovari, S. C. Dudley Jr., et al. Sudden Death in Myotonic Dystrophy N. Engl. J. Med., October 9, 2008; 359(15): 1626 - 1629. [Full Text] [PDF]

				W. J. Groh, M. R. Groh, C. Saha, J. C. Kincaid, Z. Simmons, E. Ciafaloni, R. Pourmand, R. F. Otten, D. Bhakta, G. V. Nair, et al. Electrocardiographic Abnormalities and Sudden Death in Myotonic Dystrophy Type 1 N. Engl. J. Med., June 19, 2008; 358(25): 2688 - 2697. [Abstract] [Full Text] [PDF]

				A. E. Epstein, J. P. DiMarco, K. A. Ellenbogen, N.A. M. Estes III, R. A. Freedman, L. S. Gettes, A. M. Gillinov, G. Gregoratos, S. C. Hammill, D. L. Hayes, et al. ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices) Developed in Collaboration With the American Association for Thoracic Surgery and Society of Thoracic Surgeons J. Am. Coll. Cardiol., May 27, 2008; 51(21): e1 - e62. [Full Text] [PDF]

				Writing Committee Members, A. E. Epstein, J. P. DiMarco, K. A. Ellenbogen, N.A. M. Estes III, R. A. Freedman, L. S. Gettes, A. M. Gillinov, G. Gregoratos, S. C. Hammill, et al. ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices): Developed in Collaboration With the American Association for Thoracic Surgery and Society of Thoracic Surgeons Circulation, May 27, 2008; 117(21): e350 - e408. [Full Text] [PDF]

				M. F. Nageh Recurrent fascicular ventricular tachycardia in myotonic dystrophy. Europace, January 1, 2006; 8(5): 336 - 337. [Full Text] [PDF]