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CLINICAL RESEARCH: HEART FAILURE

Improved outcomes of pediatric dilated cardiomyopathy with utilization of heart transplantation

Anna E. Tsirka, MD^*, Kathryn Trinkaus, PhD, Su-Chiung Chen, MD, Steven E. Lipshultz, MD, Jeffrey A. Towbin, MD, FACC^||, Steven D. Colan, MD, FACC^¶, Vernat Exil, MD^#, Arnold W. Strauss, MD, FACC^# and Charles E. Canter, MD, FACC^*,*

^* Department of Pediatrics, St. Louis, Missouri, USA
Department of Biostatistics, Washington University School of Medicine, St. Louis, Missouri, USA
Department of Pediatrics, St. Louis University School of Medicine, St. Louis, Missouri, USA
Department of Pediatrics, University of Miami School of Medicine, Miami, Florida, USA
^|| Department of Pediatrics, Baylor University School of Medicine, Houston, Texas, USA
^¶ Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, USA
^# Department of Pediatrics, Vanderbilt University School of Medicine, Nashville, Tennessee, USA

Manuscript received December 16, 2003; revised manuscript received February 25, 2004, accepted April 6, 2004.

^* Reprint requests and correspondence: Dr. Charles E. Canter, Division of Pediatric Cardiology, Department of Pediatrics, Washington University School of Medicine, 1 Childrens Place, St. Louis, Missouri 63110, USA.
canter{at}kids.wustl.edu

OBJECTIVES: 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.

Abbreviations and Acronyms   ACE = angiotensin-converting enzyme   CI = confidence interval   DCM = dilated cardiomyopathy   ICU = intensive care unit   IVGG = intravenous gamma globulin   LV = left ventricular   LVEDD = left ventricular end-diastolic dimension   LVEF = left ventricular ejection fraction   LVESD = left ventricular end-systolic dimension   LVSF = left ventricular shortening fraction

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