Primary prevention of sudden cardiac death in silent XXXX : role of programmed ventricular stimulation.

@entity1 @entity944 @entity299 @entity370 @entity943
BACKGROUND: Cardiac involvement in @entity370 is often silent and may lead to @entity944 . This study was designed to assess the value of programmed electric stimulation of the ventricle (PES) for risk stratification in @entity1 with @entity370 and evidence of preclinical cardiac involvement on imaging studies. METHODS AND RESULTS: @entity1 with biopsy-proven systemic @entity370 but without cardiac symptoms who had evidence of @entity299 on positron emission tomography (PET) or cardiac MRI (CMR) were included. All @entity1 underwent baseline evaluation, echocardiographic assessment of left ventricular function, and programmed electric stimulation of the ventricle. @entity1 were followed for survival and arrhythmic events. Seventy-six @entity1 underwent PES of the ventricle. Eight (11%) were inducible for sustained @entity943 and received an implantable defibrillator. None of the noninducible @entity1 received a defibrillator. Left ventricular ejection fraction was lower in @entity1 with inducible @entity943 (36.4  4.2% versus 55.8  1.5%, P<0.05). Over a median follow-up of 5 years, 6 of 8 @entity1 in the group with inducible @entity943 had @entity943 or died, compared with 1 death in the negative group (P<0.0001). CONCLUSIONS: In @entity1 with biopsy-proven @entity370 and evidence of cardiac involvement on PET or CMR alone, positive PES may help to identify @entity1 at risk for @entity943 . More importantly, @entity1 in this cohort with a negative PES appear to have a benign course within the first several years following diagnosis. PES may help to guide the use of implantable cardioverter defibrillators in this population.
