-- Mary Elizabeth Dallas
WEDNESDAY, Sept. 4 (HealthDay News) -- New research has
identified key risk factors that predict which children with a
potentially fatal heart muscle disease will face the greatest risk
of death or need for a heart transplant.
The findings could help doctors figure out which patients with
hypertrophic cardiomyopathy will benefit most from transplant
"If we knew who was in the gravest danger from this condition, we could evaluate them for early listing for heart transplant and follow them more closely," study leader Steven Lipshultz, director of the Batchelor Children's Research Institute at the University of Miami Miller School of Medicine, said in a news release.
Hypertrophic cardiomyopathy is a condition that causes the heart
muscle to thicken, which affects the ability of the heat to
function properly. People with this condition are at risk for heart
failure and sudden death.
In the study, which was to be presented Tuesday at the 2013
European Respiratory Society Annual Congress and published
simultaneously in the journal
The Lancet, researchers examined clinical and demographic
information of nearly 1,100 children diagnosed with the condition
at 98 medical centers in the United States and Canada over the
course of 19 years. They found children with inherited metabolic
diseases or hypertrophic cardiomyopathy who are also affected by
other cardiomyopathy phenotypes have a worse outlook.
The findings revealed that children who are younger at
diagnosis, as well as those who have a lower weight, congestive
heart failure and signs of abnormal left ventricle structure or
function also are at higher risk of death. The researchers said the
risk of death or heart transplant was significantly increased when
children had two or more of these risk factors at the time they
"Now we have identified patient risk factors that can be measured at the time of diagnosis to guide treatment decisions and help newly diagnosed patients and their families have some idea of what to expect from their disease," Lipshultz said. "Not only is the absolute risk of death or heart transplantation significantly higher when multiple risk factors are present, but the risk increases significantly as the number of risk factors increases."
The authors of an accompanying commentary said the findings will
be a useful tool for doctors.
"These data should allow early risk stratification and close monitoring, enabling timely and appropriate listing for heart transplantation," Dr. Robert Weintraub, from the Royal Children's Hospital in Melbourne, Australia, and Christopher Semsarian, from the Royal Prince Alfred Hospital, in Sydney. "Identification of long-term outcomes for young patients with hypertrophic cardiomyopathy, including rates of sudden death and the effect of frequently used interventions, should be a focus for future investigation."
The American Heart Association provides more on
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