WHAT: Researchers have identified important risk factors for death and transplantation in children with dilated cardiomyopathy (heart muscle disease), according to results from a study supported by the National Heart, Lung, and Blood Institute (NHLBI) of the National Institutes of Health.
Using these risk factors, physicians may be able to better identify children who will or will not benefit from life-saving transplantation surgery; the current criteria for receiving a transplant sometimes miss children who may benefit.
If the findings of the report are successfully used nationwide, the indications for heart transplantation may be broadened giving more children access to this life-prolonging therapy.
"Competing risks for death and cardiac transplantation in children with dilated cardiomyopathy: results from the Pediatric Cardiomyopathy Registry," was published online today in Circulation, the journal of the American Heart Association.
Risk factors identified for either death, transplantation, or both include presence of congestive heart failure, echocardiographic evidence of more severe disease, increased age, dilation of the left ventricle, and height. Short stature increased the risk of death but not transplantation. Researchers also found that the causes of dilated cardiomyopathy strongly affected which risk factors were predictive of death and/or transplantation. This suggests that establishing the cause of disease as soon as possible is critically important.
This population-based study used data from the Pediatric Cardiomyopathy Registry (PCMR). From 1990 to 2007, the PCMR, led by the University of Miami Leonard M. Miller School of Medicine, enrolled 1,731 children (18 years of age or younger) diagnosed with pediatric dilated cardiomyopathy, the most common heart muscle disease. Dilated cardiomyopathy can lead to heart valve problems, arrhythmias (irregular heartbeats), blood clots in the heart, and even heart failure.
For 57 percent of children who suffer from cardiomyopathy, the cause of the disease is unknown. The nationwide registry was established to collect and organize data on the condition, which will help determine the causes of the disease and lead to new and better treatments.
WHO: Jonathan R. Kaltman, M.D., medical officer in the Heart Development and Structural Diseases Branch in the Division of Cardiovascular Sciences at the NHLBI, and program officer of the NHLBI Pediatric Cardiomyopathy Registry.
Experts recommend cascade screening over universal lipid screening for familial hypercholesterolemia