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[When do you implant a pacemaker in myotonic dystrophy?].
Presse Med. 2011 Jul-Aug; 40(7-8):748-53.PM

Abstract

Myotonic dystrophy is the most frequent adult form of hereditary muscular dystrophy caused by a mutation on the DMPK gene. Myotonic dystrophy leads to multiple systemic complications related to weakness, respiratory failure, cardiac arrhythmias and cardiac conduction disturbances. Age of death is earlier in myotonic dystrophy patients than in general population with a high frequency of sudden death. Several mechanisms are involved in sudden death: atrio-ventricular block, severe ventricular arrhythmias or non-cardiac mechanism. The high degree of atrio-ventricular block is a well-recognized indication of pacemaker implantation but the prophylactic implantation of pacemaker should be considered to prevent sudden death in asymptomatic myotonic dystrophy patients. A careful clinical evaluation needs to be done for the identification of patients at high risk of sudden death. The resting ECG and SA ECG are non-invasive tools useful to select the patients who need an electrophysiologic study. In presence of prolonged HV interval more than or equal to 70 ms one can discuss the implantation of a prophylactic pacemaker. The choice of an implantable cardiac defibrillator is preferred in presence of spontaneous ventricular tachycardia or an alteration of the left ventricular ejection fraction.

Authors+Show Affiliations

Université François-Rabelais, CHU de Tours, hôpital Trousseau, 37044 Tours, France. d.babuty@chu-tours.frNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

English Abstract
Journal Article

Language

fre

PubMed ID

21549556

Citation

Babuty, Dominique, et al. "[When Do You Implant a Pacemaker in Myotonic Dystrophy?]." Presse Medicale (Paris, France : 1983), vol. 40, no. 7-8, 2011, pp. 748-53.
Babuty D, Lallemand B, Laurent V, et al. [When do you implant a pacemaker in myotonic dystrophy?]. Presse Med. 2011;40(7-8):748-53.
Babuty, D., Lallemand, B., Laurent, V., Clémenty, N., Pierre, B., Fauchier, L., Raynaud, M., & Pellieux, S. (2011). [When do you implant a pacemaker in myotonic dystrophy?]. Presse Medicale (Paris, France : 1983), 40(7-8), 748-53. https://doi.org/10.1016/j.lpm.2011.01.029
Babuty D, et al. [When Do You Implant a Pacemaker in Myotonic Dystrophy?]. Presse Med. 2011;40(7-8):748-53. PubMed PMID: 21549556.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [When do you implant a pacemaker in myotonic dystrophy?]. AU - Babuty,Dominique, AU - Lallemand,Bénédicte, AU - Laurent,Valérie, AU - Clémenty,Nicolas, AU - Pierre,Bertrand, AU - Fauchier,Laurent, AU - Raynaud,Martine, AU - Pellieux,Sybille, Y1 - 2011/05/05/ PY - 2011/01/23/received PY - 2011/01/28/accepted PY - 2011/5/10/entrez PY - 2011/5/10/pubmed PY - 2011/9/2/medline SP - 748 EP - 53 JF - Presse medicale (Paris, France : 1983) JO - Presse Med VL - 40 IS - 7-8 N2 - Myotonic dystrophy is the most frequent adult form of hereditary muscular dystrophy caused by a mutation on the DMPK gene. Myotonic dystrophy leads to multiple systemic complications related to weakness, respiratory failure, cardiac arrhythmias and cardiac conduction disturbances. Age of death is earlier in myotonic dystrophy patients than in general population with a high frequency of sudden death. Several mechanisms are involved in sudden death: atrio-ventricular block, severe ventricular arrhythmias or non-cardiac mechanism. The high degree of atrio-ventricular block is a well-recognized indication of pacemaker implantation but the prophylactic implantation of pacemaker should be considered to prevent sudden death in asymptomatic myotonic dystrophy patients. A careful clinical evaluation needs to be done for the identification of patients at high risk of sudden death. The resting ECG and SA ECG are non-invasive tools useful to select the patients who need an electrophysiologic study. In presence of prolonged HV interval more than or equal to 70 ms one can discuss the implantation of a prophylactic pacemaker. The choice of an implantable cardiac defibrillator is preferred in presence of spontaneous ventricular tachycardia or an alteration of the left ventricular ejection fraction. SN - 2213-0276 UR - https://www.unboundmedicine.com/medline/citation/21549556/[When_do_you_implant_a_pacemaker_in_myotonic_dystrophy]_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0755-4982(11)00149-7 DB - PRIME DP - Unbound Medicine ER -