Back to Search
Start Over
Risk of Arrhythmias in MYotonic Dystrophy: trial design of the RAMYD study
- Publication Year :
- 2009
-
Abstract
- Objective Myotonic dystrophy type 1 (DM1) is the most frequent muscular dystrophy in adults. DM1 is a multisystem disorder also affecting the heart with an increased incidence of sudden death, which has been explained with the common impairment of the conduction system often requiring pacemaker implantation; however, the occurrence of sudden death despite pacemaker implantation and the observation of major ventricular arrhythmias generated the hypothesis that ventricular arrhythmias may play a causal role as well. The aim of the study was to assess the 2-year cumulative incidence and the value of noninvasive and invasive findings as predictive factors for sudden death, resuscitated cardiac arrest, ventricular fibrillation, sustained ventricular tachycardia and severe sinus dysfunction or high-degree atrioventricular block. Methods/design More than 500 DM1 patients will be evaluated at baseline with a clinical interview, 12-lead ECG, 24-h ECG and echocardiogram. Conventional and nonconventional indications to electrophysiological study, pacemaker, implantable cardioverter defibrillator or loop recorder implantation have been developed. In the case of an indication to electrophysiological study, pacemaker, implantable cardioverter defibrillator or loop recorder implant at baseline or at follow-up, the patient will be referred for the procedure. At the end of 2-year follow-up, all candidate prognostic factors will be tested for their association with the endpoints. Trial registration ClinicalTrials.gov ID NCT00127582. Conclusion The available evidence supports the hypothesis that both bradyarrhythmias and tachyarrhythmias may cause sudden death in DM1, but the course of cardiac disease in DM1 is still unclear. We expect that this large, prospective, multicenter study will provide evidence to improve diagnostic and therapeutic strategies in DM1.
- Subjects :
- Tachycardia
Male
medicine.medical_specialty
Pacemaker, Artificial
Time Factors
medicine.medical_treatment
Kaplan-Meier Estimate
Sudden death
Myotonic dystrophy
Risk Assessment
Disease-Free Survival
Electrocardiography
Predictive Value of Tests
Internal medicine
medicine
Humans
Myotonic Dystrophy
cardiovascular diseases
Cardiopulmonary resuscitation
Prospective Studies
Atrioventricular Block
medicine.diagnostic_test
business.industry
Cardiac Pacing, Artificial
Arrhythmias, Cardiac
General Medicine
Implantable cardioverter-defibrillator
medicine.disease
Cardiopulmonary Resuscitation
Defibrillators, Implantable
Heart Arrest
Death, Sudden, Cardiac
Italy
Echocardiography
Research Design
Ventricular fibrillation
Ventricular Fibrillation
Cardiology
Disease Progression
Tachycardia, Ventricular
Female
Electrical conduction system of the heart
medicine.symptom
Cardiology and Cardiovascular Medicine
business
Electrophysiologic Techniques, Cardiac
Subjects
Details
- Language :
- English
- ISSN :
- 00127582
- Database :
- OpenAIRE
- Accession number :
- edsair.doi.dedup.....a9fab1fc9a05a9752c029741eddef265