Document Detail

Cardiomyopathy in children with mitochondrial disease; clinical course and cardiological findings.
MedLine Citation:
PMID:  12590906     Owner:  NLM     Status:  MEDLINE    
AIMS: To determine the frequency of cardiomyopathy in children with mitochondrial disease and describe their clinical course, prognosis and cardiological manifestations. METHODS AND RESULTS: Of 301 children with CNS and neuromuscular disease referred to our institution in 1984 to 1999, 101 had mitochondrial disease. Seventeen patients had cardiomyopathy, diagnosed by echo-Doppler investigations, all of the hypertrophic, non-obstructive type. The onset of symptomatic mitochondrial disease ranged from birth to 10 years of age. Eight children had cytochrome-c oxidase deficiency, while the remaining nine had various defects. Cardiomyopathy was diagnosed from birth to 27 years. Left ventricular posterior wall and septal thickness were both increased: z-scores +4.6+/-2.6 and +4.3+/-1.6 (mean+/-SD), respectively. The left ventricular diastolic diameter z-score, +1.3+/-3.4, and fractional shortening, 24+/-13%, displayed marked variations. Nine patients developed heart failure. Eleven patients with cardiomyopathy died, including all eight with cytochrome-c oxidase deficiency, and one patient underwent a heart transplantation. Mortality in children with mitochondrial disease was higher in those with cardiomyopathy (71%) than those without (26%) (P<0.001). CONCLUSIONS: In children with mitochondrial disease, cardiomyopathy was common (17%) and was associated with increased mortality. The prognosis for children with cytochrome-c oxidase deficiency and cardiomyopathy appeared to be particularly unfavorable.
D Holmgren; H Wåhlander; B O Eriksson; A Oldfors; E Holme; M Tulinius
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  European heart journal     Volume:  24     ISSN:  0195-668X     ISO Abbreviation:  Eur. Heart J.     Publication Date:  2003 Feb 
Date Detail:
Created Date:  2003-02-19     Completed Date:  2003-03-20     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  8006263     Medline TA:  Eur Heart J     Country:  England    
Other Details:
Languages:  eng     Pagination:  280-8     Citation Subset:  IM    
Division of Pediatric Cardiology, The Queen Silvia Children's Hospital, Göteborg, Sweden.
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MeSH Terms
Cardiomyopathy, Hypertrophic / etiology*,  pathology
Child, Preschool
Echocardiography, Doppler / methods
Electrocardiography / methods
Infant, Newborn
Mitochondrial Diseases / complications*,  pathology
Survival Analysis
Comment In:
Eur Heart J. 2003 Feb;24(3):221-4   [PMID:  12590900 ]

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