Résumé :
|
Background: Permanent pacing is recommended in patients with myotonic dystrophy type 1 (DM1) for the prevention of sudden cardiac death. However its impact on mortality is unknown.Methods: We retrospectively analyzed medical information of DM1 patients, with no prior history of pacemaker implantation, which were investigated in Pitialpi Hospital from January 2000 to December 2010. Among patients with conduction system disease on the electrocardiogram, we compared global mortality in 1/those who underwent invasive cardiac investigations, based on electrophysiological testing, and preventive permanent pacing if His to ventricle interval was >70ms, and 2/others.Results: We identified 463 patients out of 835 with conduction system disease, including 320 who underwent invasive cardiac management (age=40.7±13.3, follow up=73±36 months) and 143 who were investigated according to a conventional strategy with non-invasive cardiac tests (age=40.7±13.3, follow up=73±35 months). Global mortality was significantly reduced in patients who underwent in-depth investigations (12.5 vs. 20.2%, P=0.03). Invasive management was associated with a reduced global mortality by multivariate analysis.Conclusion: Invasive management and preventive pacing is associated with a reduced mortality in DM1 patients with conduction system disease.
|