PEDIATRICS Vol. 105 No. 6 June 2000, pp. 1260-1270
Metabolic Aspects of Myocardial Disease and a Role for L-Carnitine in the Treatment of Childhood Cardiomyopathy
Received Oct 8, 1998; accepted Aug 17, 1999.
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From * Virginia Neurological Institute, University of Virginia,
Charlottesville, Virginia; Department of Pediatric Cardiology,
Indiana University Medical Center, Indianapolis, Indiana; § Department
of Pediatric Cardiology, Geisinger Medical Center, Danville,
Pennsylvania; Division of Pediatric Cardiology, University of
Florida Health Sciences Center, Gainesville, Florida; ¶ Valley
Children's Hospital, Fresno, California; # Department of Biostatistics,
University of North Carolina, Chapel Hill, North Carolina; ** Mayo
Clinic, Rochester, Minnesota; Department of Pediatrics,
Fairview-University Medical School, University of Minnesota,
Minneapolis, Minnesota; and §§ Metabolic Research and Analysis, Inc,
Fresno, California.
Objectives. A multicenter retrospective study was conducted to investigate the possible metabolic causes of pediatric cardiomyopathy and evaluate the outcome of patients treated with L-carnitine.
Methods. Seventy-six patients diagnosed with cardiomyopathy were treated with L-carnitine in addition to conventional cardiac treatment, and 145 patients were treated with conventional treatment only. There were 101 males and 120 females between 1 day and 18 years old. Cardiomyopathy diagnoses included dilated (148 patients), hypertrophic (42 patients), restrictive (16 patients), mixed diagnosis (11 patients), and 4 with an unknown type. Of 76 L-carnitine-treated patients, 29 (38%) had evidence to suggest a disorder of metabolism, and of 145 control patients, 15 (10%) were suspected to have a disorder of metabolism. These metabolic disorders were thought to be the cause for the cardiomyopathy of the patients. The duration of L-carnitine treatment ranged from 2 weeks to >1 year. Information was collected on length of survival (time-to-event), clinical outcome, echocardiogram parameters, and clinical assessments. Data were collected at intervals from baseline to study endpoint, death, transplant, or last known follow-up visit.
Results. L-Carnitine-treated patients were younger than control patients and had poorer clinical functioning at baseline, yet they demonstrated lower mortality and a level of clinical functioning and clinical severity comparable to control patients on conventional therapy by the end of the study. An analysis of the interaction between clinical outcome and concomitant medications unexpectedly revealed that the population of patients treated with angiotensin-converting enzyme (ACE) inhibitors (40% of patients) had significantly poorer survival (although their greater likelihood for poor survival may possibly have made them more likely to receive ACE inhibitors).
Conclusion. Results suggest that L-carnitine provides clinical benefit in treating pediatric cardiomyopathy. There is a need for further exploration of potential explanatory factors for the higher mortality observed in the population of patients treated with ACE inhibitors. Key words: pediatric, cardiology, cardiomyopathy, L-carnitine, metabolism, genetics.
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