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JAMA. 2012 Mar 28;307(12):1292-301. doi: 10.1001/jama.2012.346.

Electrophysiological study with prophylactic pacing and survival in adults with myotonic dystrophy and conduction system disease.

Author information

  • 1Pierre et Marie Curie-Paris 6 University, Myology Institute, Pitié-Salpêtrière Hospital, 75013 Paris, France. karim.wahbi@cch.aphp.fr

Erratum in

  • JAMA. 2012 Jul 25;308(4):342.

Abstract

CONTEXT:

Up to one-third of patients with myotonic dystrophy type 1 die suddenly. Thus far, no intervention has effectively prevented sudden death.

OBJECTIVE:

To determine whether an invasive strategy based on systematic electrophysiological studies and prophylactic permanent pacing is associated with longer survival in patients presenting with myotonic dystrophy type 1 and major infranodal conduction delays than a noninvasive strategy.

DESIGN, SETTING, AND PATIENTS:

A retrospective study, the DM1 Heart Registry included 914 consecutive patients older than 18 years with genetically confirmed myotonic dystrophy type 1 who were admitted to the Neurological Unit of the Myology Institute of Pitié-Salpêtrière Hospital, a teaching medical center in Paris, France, between January 2000 and December 2009.

INTERVENTIONS:

Among 486 patients whose electrocardiogram showed a PR interval greater than 200 milliseconds, a QRS duration greater than 100 milliseconds, or both, the outcome of 341 (70.2%) who underwent an invasive strategy was compared with 145 (29.8%) who underwent a noninvasive strategy. A propensity score risk adjustment and propensity-based matching analysis was used to account for selection biases.

MAIN OUTCOME MEASURES:

Rates of overall survival (main outcome measure) and sudden death, respiratory death, and other deaths (secondary outcome measures).

RESULTS:

Over a median follow-up of 7.4 years (range, 0-9.9 years), 50 patients died in the invasive strategy group and 30 died in the noninvasive strategy group (hazard ratio [HR], 0.74 [95 CI, 0.47-1.16]; P = .19), corresponding to an overall 9-year survival of 74.4% (95% CI, 69.2%-79.9%). Regardless of the technique used to adjust for between-group differences in baseline characteristics, the invasive strategy was associated with a longer survival, with adjusted HRs ranging from 0.47 (95% CI, 0.26-0.84; P = .01) for a covariate-adjusted analysis of propensity-matched data to 0.61 (95% CI, 0.38-0.99; P = .047) for an analysis adjusted for propensity score quintiles. The survival difference was largely attributable to a lower incidence of sudden death, which occurred in 10 patients in the invasive strategy group and in 16 patients in the noninvasive strategy group, with HRs ranging from 0.24 (95% CI, 0.10-0.56; P = .001) for an analysis adjusted for propensity score quintiles and covariates to 0.28 (95% CI, 0.13-0.61; P = .001) for an unadjusted analysis of propensity-matched data.

CONCLUSION:

Among patients with myotonic dystrophy type 1, an invasive strategy was associated with a higher rate of 9-year survival than a noninvasive strategy.

TRIAL REGISTRATION:

clinicaltrials.gov Identifier: NCT01136330.

Comment in

PMID:
22453570
[PubMed - indexed for MEDLINE]
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