Clinical features and outcomes of childhood dilated cardiomyopathy: results from a national population-based study.
BACKGROUND
Despite considerable mortality, population-based prognostic factors for childhood dilated cardiomyopathy are lacking.


RESULTS
A population-based cohort study was undertaken of all children in Australia who presented with cardiomyopathy at age 0 to 10 years between January 1, 1987, and December 31, 1996. A single cardiologist analyzed all cardiac investigations, and a single pathologist analyzed histopathological material. There were 184 subjects with dilated cardiomyopathy. Positive viral identification or lymphocytic myocarditis was found in 30 (68.2%) of 44 cases with available early histology and 8 of 9 cases presenting with sudden death. Freedom from death or transplantation was 72% (95% CI, 65% to 78%) 1 year after presentation and 63% (95% CI, 55% to 70%) at 5 years. By proportional hazards regression analysis, risk factors for death or transplantation comprised age >5 years at presentation (hazard ratio 5.6, 95% CI, 2.6 to 12.0), familial dilated cardiomyopathy (hazard ratio, 2.9; 95% CI, 1.5 to 5.6), lower initial fractional shortening z score (hazard ratio per z-score unit, 0.75; 95% CI, 0.65 to 0.87), and failure to increase fractional shortening z score during follow-up (hazard ratio per unit increase, 0.68; 95% CI, 0.58 to 0.79). At follow-up, 78 (44.6%) of 175 cases diagnosed during life have no symptoms and are not taking any cardiac medication.


CONCLUSIONS
Early mortality is high in childhood dilated cardiomyopathy, but the clinical status of long-term survivors is good. This population-based study identifies children at risk of adverse events.
Daubeney
Piers E F
PE
Department of Cardiology, Royal Children's Hospital, Melbourne, Australia.
Nugent
Alan W
AW
Chondros
Patty
P
Carlin
John B
JB
Colan
Steven D
SD
Cheung
Michael
M
Davis
Andrew M
AM
Chow
C W
CW
Weintraub
Robert G
RG
D002311:Cardiomyopathy, Dilated / Q000209:etiology / Q000235:genetics / Q000401:mortality* / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D003241:Consanguinity; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009205:Myocarditis / Q000150:complications; D011379:Prognosis