CONTEXT: Up to one-third of @entity1 with @entity3918 type 1 die suddenly. Thus far, no intervention has effectively prevented @entity944 . OBJECTIVE: To determine whether an invasive strategy based on systematic electrophysiological studies and prophylactic permanent pacing is associated with longer survival in @entity1 presenting with @entity3918 type 1 and major infranodal conduction delays than a noninvasive strategy. DESIGN, SETTING, AND @entity1 : A retrospective study, the @entity5471 Heart Registry included 914 consecutive @entity1 older than 18 years with genetically confirmed @entity3918 type 1 who were admitted to the Neurological Unit of the Myology Institute of Piti  - @entity9574  tri  re Hospital, a teaching medical center in Paris, France, between January 2000 and December 2009. INTERVENTIONS: Among 486 @entity1 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 @entity944 , @entity204 , and other deaths (secondary outcome measures). RESULTS: Over a median follow-up of 7.4 years (range, 0-9.9 years), 50 @entity1 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 @entity944 , which occurred in 10 @entity1 in the invasive strategy group and in 16 @entity1 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 @entity1 with @entity3918 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.

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

@entity1 :: ('9606', 'Species') :: ['patients', 'PATIENTS'] @entity9574 :: ('10656', 'Gene') :: ['Salp'] @entity944 :: ('MESH:D003645', 'Disease') :: ['sudden death'] @entity5471 :: ('28509', 'Gene') :: ['DM1'] @entity3918 :: ('MESH:D009223', 'Disease') :: ['myotonic dystrophy'] @entity204 :: ('MESH:D003643', 'Disease') :: ['respiratory death']
@entity3918 :: (MESH:D009223,Disease) :: ['myotonic dystrophy']
