BACKGROUND: Population-based studies have provided insight into the natural history of adult @entity1121 , but comparable information for affected @entity1 is lacking. METHODS AND RESULTS: All Australian @entity1 who presented with primary @entity296 at 0 to 10 years of age between January 1, 1987, and December 31, 1996, were enrolled in a longitudinal cohort study. A single cardiologist reviewed serial cardiac investigations on each subject. A total of 80 subjects with @entity1121 were identified. An underlying syndromal, genetic, or metabolic condition was identified in 46 subjects (57.5%). There were no cases of @entity944 at presentation. @entity3585 was present in 32 subjects (40%); right @entity3585 was present in 10 (12.5%). Freedom from @entity204 or transplantation was 83% (95% CI, 73 to 90) 5 years after presentation and 76% (95% CI, 62 to 86) 10 years after presentation. By proportional-hazards regression analysis, risk factors for @entity204 or transplantation included @entity4346 , age at presentation <1 year, lower initial fractional shortening Z score, and increasing left ventricular posterior wall thickness relative to body surface area. At the latest follow-up, 54 of 65 surviving subjects had no symptoms, and 46 were receiving no regular medication. CONCLUSIONS: Syndromal, genetic, and metabolic causes predominate in @entity1 with @entity1121 . Ventricular outflow tract obstruction is common. The clinical status of long-term survivors is good. This population-based study identifies @entity1 with @entity1121 who are at risk of adverse events.

Clinical features and outcomes of childhood XXXX : results from a national population-based study.

@entity1 :: ('9606', 'Species') :: ['children'] @entity296 :: ('MESH:D009202', 'Disease') :: ['cardiomyopathy'] @entity3585 :: ('MESH:D014694', 'Disease') :: ['Left ventricular outflow tract obstruction', 'ventricular outflow obstruction'] @entity944 :: ('MESH:D003645', 'Disease') :: ['sudden death'] @entity4346 :: ('MESH:D017379', 'Disease') :: ['concentric left ventricular hypertrophy'] @entity1121 :: ('MESH:D002312', 'Disease') :: ['hypertrophic cardiomyopathy'] @entity204 :: ('MESH:D003643', 'Disease') :: ['death']
@entity1121 :: (MESH:D002312,Disease) :: ['hypertrophic cardiomyopathy']
