Frequency of cardiac death in XXXX with @entity1000 .

@entity1 @entity1164 @entity1165 @entity1000 @entity2224 @entity943
The prognosis in @entity1 with @entity1000 ( @entity1000 ) is guarded, with the 5-year mortality rate ranging from 14% to 50%, owing to @entity1164 ( @entity1164 ) or pump failure. The risk factors for @entity1164 in adults with @entity1000 include asymptomatic @entity1165 and poor left ventricular function. It is unclear whether these findings can be extrapolated to the pediatric population. A retrospective review of all @entity1 with the diagnosis of @entity1000 seen at a single institution from 1990 to 2004 was performed. A total of 85 @entity1 (46 males), with a median age of 3.8 years (0 days to 17.3 years) were studied. The mean left ventricular ejection fraction was 25 +/- 12% (median 23%, range 45% to 45%) at presentation. The following @entity943 occurred at presentation or during the initial hospitalization: @entity1165 in 6, sustained @entity2224 in 1, @entity943 in 6, and both @entity943 in 1. During a subsequent hospitalization or outpatient follow-up, 7 @entity1 had the following @entity943 : @entity943 in 2, @entity1165 in 4, and both @entity943 in 1. The cumulative survival rate was 40% at a mean follow-up of 6.2 years (95% confidence interval 4.4 to 8.1). One single episode of @entity1164 occurred in 1 @entity1 without a history of sustained @entity943 . In conclusion, in @entity1 with @entity1000 , despite the low left ventricular ejection fraction and presence of @entity943 , only one episode of @entity1164 occurred in this group of @entity1 . Given the 1% incidence of @entity1164 in this cohort, the use of implantable cardioverter-defibrillators as primary prevention in @entity1 with @entity1000 might not be indicated.
