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Children with heart failure from dilated cardiomyopathy are seeing a dramatic improvement in outcomes in recent years

Date:
February 5, 2018
Source:
Children's Hospital of Michigan
Summary:
New research has shown that for children with heart failure from dilated cardiomyopathy there has been a dramatic improvement in outcomes of medical management in the past few years. The study also shows that significantly fewer of these patients die from heart disease.
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A multi-center initiative involving 98 centers across the U.S. and Canada, conducted by the National Institutes of Health-supported Pediatric Cardiomyopathy Registry, has shown that for children with heart failure from dilated cardiomyopathy there has been a dramatic improvement in outcomes of medical management in the past few years. The study also shows that significantly fewer of these patients die from heart disease.

Results from this international study show for the first time improved transplant-free survival rates for pediatric dilated cardiomyopathy patients. The important findings appeared in an article titled "Survival without Cardiac Transplantation Among Children with Dilated Cardiomyopathy," which were featured in the November 28, 2017 issue of the Journal of the American College of Cardiology.

The study, was conducted through The Pediatric Cardiomyopathy Registry, a National Heart, Lung, and Blood Institute and Children's Cardiomyopathy Foundation sponsored registry. It was created to study the outcomes of children with various heart muscle disorders known as cardiomyopathies.

Steven Lipshultz, MD, and his colleagues founded the Pediatric Cardiomyopathy Registry, in 1990. Dr. Lipshultz is interim director of the Children's Research Center of Michigan at the DMC Children's Hospital of Michigan, Carman and Ann Adams Endowed Chair in Pediatric Research at the Wayne State University School of Medicine, and senior author of the study. The Children's Research Center of Michigan at the DMC Children's Hospital of Michigan receives significant infrastructure support from the Children's Hospital of Michigan Foundation that facilitates these research discoveries.

Cardiomyopathy is a heart condition involving abnormalities of the muscle fibers, which contract with each heartbeat. According to the Pediatric Cardiomyopathy Registry, one in every 100,000 children in the U.S. under the age of 18 is diagnosed with cardiomyopathy. Dilated cardiomyopathy is the most common type of cardiomyopathy in infancy, childhood, and adolescence.

For this study, outcomes of 1,199 children diagnosed with dilated cardiomyopathy from 1990-1999 were compared with 754 children diagnosed with dilated cardiomyopathy from 2000-2009. The study concluded that, even though the percentage of patients undergoing heart transplants remained constant, children with dilated cardiomyopathy in the latter decade had significantly higher survival rates and were living longer without a transplant.

Dr. Lipshultz says although dilated cardiomyopathy is a relatively rare disease in children,nearly 40% of children with dilated cardiomyopathy require a heart transplant or die within 2 years of diagnosis. Heart transplantation has improved the outcomes of children with dilated cardiomyopathy but is limited by donor heart availability. Newer therapies, including new medications, advanced ICU care and artificial heart machines, and comprehensive chronic illness management are now being used to treat children with dilated cardiomyopathy. The study investigated whether children with dilated cardiomyopathy were surviving longer without the need for heart transplantation.

Dr. Lipshultz says this study showcases a remarkable accomplishment demonstrating the value of leading pediatric centers throughout North America gathering information and sharing best practices to improve outcomes.

"We have been able to reduce failure of medical management by about 50% which translates to a large number of children with dilated cardiomyopathy who are alive without heart transplant compared to children with dilated cardiomyopathy at the same centers in the past who would have died. When you consider that in this population half the children who would have died are living this is truly incredible," he says.

"Researchers believe the most likely reasons for this improvement could be improved medical therapy or increased use of mechanical support devices," says Dr. Lipshultz. "This ground breaking study is the first report to show improved transplant-free survival for pediatric dilated cardiomyopathy patients followed by the Pediatric Cardiomyopathy Registry. Discovering how we can have these children reach better outcomes such as by documentation of best medical therapy and use of mechanical circulatory support, and then implementing these clinical practice guidelines to achieve these better outcomes, may lead to hundreds of children not needing a heart transplant or possibly preventing cardiac death," he adds.

Lisa Yue, the founding executive director of the Children's Cardiomyopathy Foundation, which funded the study with the National Institutes of Health, says "the study shows how collaboration among research centers and funding organizations can lead to improved medical management and patient outcomes." Mrs. Yue and her husband Eddie Yu lost two of their children to cardiomyopathy and formed the Foundation to advance research on pediatric cardiomyopathy.

"After working with Dr. Lipshultz and the Pediatric Cardiomyopathy Registry for so many years, it's extremely encouraging that children with severe cardiomyopathy are twice as likely to be living now, even without a transplant," says Mrs. Yue.


Story Source:

Materials provided by Children's Hospital of Michigan. Note: Content may be edited for style and length.


Journal Reference:

  1. Rakesh K. Singh, Charles E. Canter, Ling Shi, Steven D. Colan, Debra A. Dodd, Melanie D. Everitt, Daphne T. Hsu, John L. Jefferies, Paul F. Kantor, Elfriede Pahl, Joseph W. Rossano, Jeffrey A. Towbin, James D. Wilkinson, Steven E. Lipshultz. Survival Without Cardiac Transplantation Among Children With Dilated Cardiomyopathy. Journal of the American College of Cardiology, 2017; 70 (21): 2663 DOI: 10.1016/j.jacc.2017.09.1089

Cite This Page:

Children's Hospital of Michigan. "Children with heart failure from dilated cardiomyopathy are seeing a dramatic improvement in outcomes in recent years." ScienceDaily. ScienceDaily, 5 February 2018. <www.sciencedaily.com/releases/2018/02/180205161639.htm>.
Children's Hospital of Michigan. (2018, February 5). Children with heart failure from dilated cardiomyopathy are seeing a dramatic improvement in outcomes in recent years. ScienceDaily. Retrieved March 27, 2024 from www.sciencedaily.com/releases/2018/02/180205161639.htm
Children's Hospital of Michigan. "Children with heart failure from dilated cardiomyopathy are seeing a dramatic improvement in outcomes in recent years." ScienceDaily. www.sciencedaily.com/releases/2018/02/180205161639.htm (accessed March 27, 2024).

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