Scientists funded by the British Heart Foundation have developed the first ever tool to identify children at risk of sudden death from hypertrophic cardiomyopathy (HCM).
HCM is an inherited heart condition that causes the heart muscle to become thicker. It can lead to life-threatening abnormal heart rhythms and sudden cardiac arrest, which is the leading cause of death in children with the condition. Most people with HCM have few, if any, symptoms. Although there are tools which can help predict the risk of sudden death in adults with the condition, until now they haven’t existed for children.
Scientists at University College London and Great Ormond Street Hospital collected anonymised medical records from cardiac centers around the world to find out which factors are associated with a higher risk of sudden death in children with HCM. They used this to develop a tool that means doctors can identify children with HCM who may need to be fitted with an implantable cardioverter defibrillator (ICD) which can shock the heart back into a normal rhythm if they experience a life-threatening abnormal heart rhythm. The new tool could also help to reassure families of those children with HCM who are found to be unlikely to experience a cardiac arrest.
Professor Jeremy Pearson, Associate Medical Director at the British Heart Foundation, which part-funded the research, said:
“No parent should have to go through losing their child to HCM. By fine-tuning doctors’ ability to spot which children with this potentially deadly condition are most at risk of having a cardiac arrest, this discovery should save lives.
“HCM is inherited, and can devastate whole families before the condition is identified. Most people who have HCM are undiagnosed and suffer few, if any, symptoms. BHF research has uncovered many of the faulty genes underlying this deadly heart condition, enabling genetic screening so it can be spotted and people can receive the best treatments available.
“This research shows that we can now more effectively identify children at risk of a cardiac arrest from HCM and who will benefit from an implantable defibrillator. However, there is more to be done. Only through more research can we find new drugs and therapies to improve the lives of children with living with life-threatening heart conditions.”
SOURCE: British Heart Foundation