title: Treating children with idiopathic dilated cardiomyopathy (from the Pediatric Cardiomyopathy Registry).
abstract: In 40% of children with symptomatic idiopathic dilated cardiomyopathy (IDC), medical therapy fails within 2 years of diagnosis. Strong evidence-based therapies are not available for these children, and how evidence-based therapies for adults with IDC should be applied to children is unclear. Using data from the National Heart, Lung, and Blood Institute's Pediatric Cardiomyopathy Registry, we compared practice patterns of initial therapies for children with IDC diagnosed from 1990 to 1995 (n = 350) and from 2000 to 2006 (n = 219). At diagnosis, 73% had symptomatic heart failure (HF), and 7% had > or =1 family member with IDC. Anti-HF medications were most commonly prescribed initially. Anti-HF medication use was similar across the 2 periods (84% and 87%, respectively), as was angiotensin-converting enzyme inhibitor use (66% and 70%, respectively). These medications were used more commonly in children with greater left ventricular dilation and poorer left ventricular fractional shortening and functional class (p <0.001). Beta-blocker use was 4% to 18% over the 2 periods. Treatments for pediatric IDC have changed little over the previous 25 years. Anti-HF medications remain the most common treatment, and they are often given to children with asymptomatic left ventricular dysfunction. Children with asymptomatic left ventricular dysfunction are often not offered angiotensin-converting enzyme inhibitors without echocardiographic evidence of advanced disease. In conclusion, therapeutic clinical trials are strongly indicated because practice variation is substantial and medical outcomes in these children have not improved in the previous several decades.
authors: Department of Pediatrics, University of Miami, Leonard M. Miller School of Medicine, Miami, Florida, USA.
authors: William G
authors: WG
authors: Harmon
authors: Lynn A
authors: LA
authors: Sleeper
authors: Leigha
authors: L
authors: Cuniberti
authors: Jane
authors: J
authors: Messere
authors: Steven D
authors: SD
authors: Colan
authors: E John
authors: EJ
authors: Orav
authors: Jeffrey A
authors: JA
authors: Towbin
authors: James D
authors: JD
authors: Wilkinson
authors: Steven E
authors: SE
authors: Lipshultz
mesh_terms: D000293:Adolescent; D002311:Cardiomyopathy, Dilated / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy*; D002316:Cardiotonic Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D016001:Confidence Intervals; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D016015:Logistic Models; D008297:Male; D015999:Multivariate Analysis; D016017:Odds Ratio; D011446:Prospective Studies; D012042:Registries
chemical_list: D002316:Cardiotonic Agents