Improved outcomes of pediatric dilated cardiomyopathy with utilization of heart transplantation.
OBJECTIVE
We studied the outcomes of pediatric patients diagnosed with dilated cardiomyopathy (DCM) and their relation to epidemiologic and echocardiographic variables at the time of presentation.


BACKGROUND
The outcome of pediatric DCM patients ranges from recovery to a 50% to 60% chance of death within five years of diagnosis. The impact of heart transplantation and other emerging therapies on the outcomes of pediatric DCM patients is uncertain.


METHODS
We performed a retrospective study of the outcomes in 91 pediatric patients diagnosed with DCM from 1990 to 1999. Routine therapy included use of digoxin, diuretics, angiotensin-converting enzyme inhibitors, and heart transplantation.


RESULTS
At the time of last follow-up, 11 patients (12%) had died without transplantation; 20 (22%) underwent transplantation; 27 (30%) had persistent cardiomyopathy; and 33 (36%) had recovery of left ventricular systolic function. Overall actuarial one-year survival was 90%, and five-year survival was 83%. However, actuarial freedom from "heart death" (death or transplantation) was only 70% at one year and 58% at five years. Multivariate analysis found age <1 year (hazard ratio 7.1), age >12 years (hazard ratio 4.5), and female gender (hazard ratio 3.0) to be significantly associated with a greater risk of death or transplantation and a higher left ventricular shortening fraction at presentation (hazard ratio 0.92), with a slightly decreased risk of death or transplantation.


CONCLUSIONS
Pediatric DCM patients continue to have multiple outcomes, with recovery of left ventricular systolic function occurring most frequently. Utilization of heart transplantation has led to improved survival after the diagnosis of pediatric DCM.
Tsirka
Anna E
AE
Department of Pediatrics, Washington University School of Medicine, St. Louis, Missouri, USA.
Trinkaus
Kathryn
K
Chen
Su-Chiung
SC
Lipshultz
Steven E
SE
Towbin
Jeffrey A
JA
Colan
Steven D
SD
Exil
Vernat
V
Strauss
Arnold W
AW
Canter
Charles E
CE
D000293:Adolescent; D002311:Cardiomyopathy, Dilated / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D016027:Heart Transplantation / Q000401:mortality; D006801:Humans; D007223:Infant; D008297:Male; D015996:Survival Rate; D016896:Treatment Outcome; D016277:Ventricular Function, Left