TY - JOUR
T1 - Clinical features and outcomes of childhood dilated cardiomyopathy
T2 - Results from a national population-based study
AU - Daubeney, Piers E F
AU - Nugent, Alan W.
AU - Chondros, Patty
AU - Carlin, John B.
AU - Colan, Steven D.
AU - Cheung, Michael
AU - Davis, Andrew M.
AU - Chow, C. W.
AU - Weintraub, Robert G.
PY - 2006/12
Y1 - 2006/12
N2 - BACKGROUND - Despite considerable mortality, population-based prognostic factors for childhood dilated cardiomyopathy are lacking. METHODS AND 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.
AB - BACKGROUND - Despite considerable mortality, population-based prognostic factors for childhood dilated cardiomyopathy are lacking. METHODS AND 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.
KW - Cardiomyopathy
KW - Heart failure
KW - Myocarditis
KW - Pediatrics
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U2 - 10.1161/CIRCULATIONAHA.106.635128
DO - 10.1161/CIRCULATIONAHA.106.635128
M3 - Article
C2 - 17116768
AN - SCOPUS:33845720299
SN - 0009-7322
VL - 114
SP - 2671
EP - 2678
JO - Circulation
JF - Circulation
IS - 24
ER -