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The impact of changing medical therapy on transplantation-free survival in pediatric dilated cardiomyopathy.

Abstract：

OBJECTIVES:We sought to determine whether the introduction of these agents had altered the outcome of dilated cardiomyopathy (DC) in childhood. BACKGROUND:Pediatric DC has a poor prognosis. Angiotensin-converting enzyme inhibitors (ACEIs) and beta-adrenergic receptor blockers (BBs) improve survival in adults with DC, but their effectiveness in children has not been confirmed. METHODS:We performed a single-institution retrospective review of all diagnosed cases of DC and related phenotypic variants between 1976 and 2005, with multivariate analysis of risk factors for the end point of death or cardiac transplantation. RESULTS:A total of 189 patients presented between January 1, 1976, and March 31, 2005. Forty-four patients died, 34 underwent cardiac transplantation, and 10 were lost to follow-up during this period. The 2- and 5-year transplantation-free survival rates for all patients were 63.6% (95% confidence interval [CI]: 56.4% to 70.8%) and 56.3% (95% CI: 48.5% to 64.1%), respectively. For patients treated with digoxin but neither an ACEI nor a BB (n = 51), the 5-year transplantation free survival rate was 67.5% (95% CI: 53.5% to 82.0%) and for those treated with the addition of an ACEI but no BB (n = 65), the rate was 57.2% (95% CI: 43.6% to 69.4%) (p = NS). Combination therapy with an ACEI and a BB (n = 57) was not associated with an improvement in 5-year transplantation-free survival (58.5%; 95% CI: 42.5% to 72.0%, p = NS). In multivariable analysis, presentation with a low left ventricular ejection fraction increased the risk of death or transplantation, but the end point was not influenced by time era or treatment strategy. CONCLUSIONS:DC in childhood has a high risk of death or the need for transplantation. Medical treatment has shifted toward combination ACEI and BB therapy in the current era. Our retrospective data, however, suggest only a transient survival advantage associated with the combined use of ACEI and BB over ACEI alone and no obvious or sustained improvement in transplantation-free survival accompanying the change from digoxin-based medical therapy.

journal_name

J Am Coll Cardiol

journal_title

Journal of the American College of Cardiology

authors

Kantor PF,Abraham JR,Dipchand AI,Benson LN,Redington AN

doi

10.1016/j.jacc.2009.11.059

subject

Has Abstract

pub_date

2010-03-30 00:00:00

pages

1377-84

issue

eissn

0735-1097

issn

1558-3597

pii

S0735-1097(10)00274-3

journal_volume

pub_type

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The impact of changing medical therapy on transplantation-free survival in pediatric dilated cardiomyopathy.