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Characteristics and outcomes of cardiomyopathy in children with Duchenne or Becker muscular dystrophy: a comparative study from the Pediatric Cardiomyopathy Registry.

Abstract：

OBJECTIVE:The aim of this study was to determine in pediatric Duchenne (DMD) and Becker muscular dystrophy (BMD) or other dilated cardiomyopathies (ODCM) whether outcomes differ by diagnosis. BACKGROUND:Children with dilated cardiomyopathy are treated as a single undifferentiated group. METHODS:This cohort study of 128 children with DMD, 15 with BMD, and 312 with ODCM uses outcome measures of left ventricular (LV) size and function, death, heart transplant, and death or transplant. RESULTS:At cardiomyopathy diagnosis, the DMD and BMD groups had similar mean ages (14.4 and 14.6 years), prevalence of congestive heart failure (CHF) (30% and 33%), and LV fractional shortening (FS) Z-scores (median, -5.2 for DMD and -6.7 for BMD). The BMD group had more severe mitral regurgitation (P = .05) and a higher mean LV end-diastolic dimension Z-score than the DMD group (2.9 +/- 1.5 vs 1.2 +/- 1.9, P = .002). Duchenne muscular dystrophy group survival was lower than in BMD or ODCM groups (P = .06) at 5 years (57%, 100%, and 71%, respectively). In BMD, 25% received cardiac transplants within 0.4 years of cardiomyopathy diagnosis. The combined DMD and BMD group had less LV dilation and a closer-to-normal LV FS at cardiomyopathy diagnosis than the ODCM group. After 2 years, LV dilation increased, and LV FS did not change in the combined DMD and BMD group; for ODCM patients, LV dilation did not progress, and LV FS improved. CONCLUSIONS:Children with DMD and cardiomyopathy have a higher mortality. Becker muscular dystrophy has a high heart transplantation rate in the 5 years after diagnosis of cardiomyopathy. Serial echocardiography demonstrates a different disease course for DMD and BMD patients compared with ODCM patients.

journal_name

Am Heart J

journal_title

American heart journal

authors

Connuck DM,Sleeper LA,Colan SD,Cox GF,Towbin JA,Lowe AM,Wilkinson JD,Orav EJ,Cuniberti L,Salbert BA,Lipshultz SE,Pediatric Cardiomyopathy Registry Study Group.

doi

10.1016/j.ahj.2008.01.018

subject

Has Abstract

pub_date

2008-06-01 00:00:00

pages

998-1005

issue

eissn

0002-8703

issn

1097-6744

pii

S0002-8703(08)00064-1

journal_volume

155

pub_type

杂志文章

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Characteristics and outcomes of cardiomyopathy in children with Duchenne or Becker muscular dystrophy: a comparative study from the Pediatric Cardiomyopathy Registry.