OBJECTIVE: To identify determinants of survival by comparing cardiopulmonary function in two @entity1 groups: prolonged survivors of @entity1555 ( @entity1555 ) versus @entity1555 @entity1 who experienced @entity204 ( @entity204 ). METHODS: Retrospective chart review of our @entity1555 @entity1 from 1999 to 2013. @entity1162 ( @entity1162 ) was defined as alive and >= 30 years old. @entity204 ( @entity204 ) was defined as @entity204 at < 30 years old. EXCLUSION CRITERIA: @entity479 therapy. RESULTS: Eleven @entity1 met criteria for @entity1162 and 14 @entity1 for @entity204 (mean age        SD: 34.3        4.3 years vs. 21.7        3.8 years, respectively; P   <   0.001). Pulmonary function was better in the @entity204 @entity1 : all @entity1162 @entity1 had a vital capacity of 0   ml (n   =   11) versus 23% (3/13) of the @entity204 @entity1 (P   <   0.001). Thirteen of 14 @entity204 @entity1 and all @entity1162 @entity1 received assisted ventilation. Heart function was worse in the @entity204 @entity1 : ejection fraction (EF) was 42.2        14.2% in the @entity1162 @entity1 (n   =   11) versus 29.2        14.1% in the @entity204 @entity1 (n   =   13; P   =   0.035). @entity384 was present in 36% (4/11) of @entity1162 @entity1 versus 78% (11/14) of @entity204 @entity1 (P   =0.048). Among @entity204 @entity1 , 57% (8/14) died from progressive @entity296 . CONCLUSION: In our study group, good heart function was a pre-condition for @entity1162 and poor heart function was the primary cause of @entity204 . Our results suggest that, when @entity1555 @entity1 are treated with assisted ventilation, heart function is the main determinant of their survival. Pediatr Pulmonol.    2015 Wiley Periodicals, Inc.

Cardiac phenotype determines survival in XXXX .

@entity1 :: ('9606', 'Species') :: ['patients', 'patient'] @entity296 :: ('MESH:D009202', 'Disease') :: ['cardiomyopathy'] @entity384 :: ('MESH:D002311', 'Disease') :: ['Dilated cardiomyopathy'] @entity1555 :: ('MESH:D020388', 'Disease') :: ['Duchenne muscular dystrophy', 'DMD'] @entity1162 :: ('MESH:D008133', 'Disease') :: ['PS', 'Prolonged Survival'] @entity204 :: ('MESH:D003643', 'Disease') :: ['ED', 'early death', 'Early death', 'death'] @entity479 :: ('D013256', 'Chemical') :: ['steroid']
@entity1555 :: (MESH:D020388,Disease) :: ['Duchenne muscular dystrophy']
