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Invasive Strategy Ups Survival in Myotonic Dystrophy 1

Longer survival with invasive strategy based on prophylactic permanent pacing

Invasive Strategy Ups Survival in Myotonic Dystrophy 1

WEDNESDAY, March 28 (HealthDay News) -- An invasive strategy, based on prophylactic permanent pacing, is associated with longer survival for patients with myotonic dystrophy type 1, according to a study published in the March 28 issue of the Journal of the American Medical Association.

Karim Wahbi, M.D., of Pitié-Saltpêtière Hospital in Paris, and colleagues conducted a retrospective study of 914 consecutive patients (older than 18 years) with genetically confirmed myotonic dystrophy type 1 who were admitted to the hospital from 2000 through 2009. Of the 486 patients whose electrocardiogram showed a PR interval greater than 200 milliseconds or a QRS duration greater than 100 milliseconds, or both, 70.2 percent underwent an invasive treatment strategy based on systematic electrophysiological studies and prophylactic permanent pacing and 29.8 percent underwent a noninvasive strategy.

During a median of 7.4 years of follow-up, the researchers found that 50 patients in the invasive strategy group and 30 in the noninvasive strategy group died (hazard ratio [HR], 0.74; P = 0.19), corresponding to an overall nine-year survival of 74.4 percent. Adjusting for between-group differences in baseline characteristics, the invasive strategy was associated with significantly longer survival, with adjusted HRs ranging from 0.47 to 0.61. The survival difference was mainly due to a reduced incidence of sudden death, which occurred in 10 patients in the invasive strategy group versus 16 patients in the noninvasive strategy group (HRs ranging from 0.24 to 0.28).

"Among patients with myotonic dystrophy type 1, an invasive strategy was associated with a higher rate of nine-year survival than a noninvasive strategy," the authors write.

One author disclosed financial ties to the medical device industry; one author disclosed financial ties to Genzyme.

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