Young children with cardiomyopathy at low risk of dying suddenly

2nd June 2015

Researchers have been looking into the risks of young children dying suddenly from cardiomyopathy.

Though the disease can be serious in young children and difficult to treat, during a median follow-up of 12 years they found only around one in 20 had died suddenly, the researchers reported in the Journal of the American College of Cardiology.

A total of 289 children aged under ten were enrolled in the National Australian Childhood Cardiomyopathy Study. The on-going study is assessing all children diagnosed in the country with cardiomyopathy from 1987 to 1996.

Each child was categorised as having dilated cardiomyopathy, hypertrophic cardiomyopathy, restrictive cardiomyopathy or left ventricular noncompaction.

In the 12 year follow-up, 5.5 per cent of the children died suddenly, and risk varied depending on the type of cardiomyopathy.

At 15 years, those with non-compaction were twice as likely to die suddenly as those with restrictive cardiomyopathy, almost four times as likely as those with hypertrophic cardiomyopathy and almost five times as those with dilated cardiomyopathy.

The researchers said risk factors also included older age at diagnosis, a family history of cardiomyopathy and severity of left ventricle dysfunction.

A higher posterior wall thickness was the sole risk factor identified for children with hypertrophic cardiomyopathy, the researchers said.

They concluded that larger childhood studies of individual cardiomyopathy types might be helpful for identificating children most at risk.

The study is continuing to follow the children into adulthood.

The study is supported by the Royal Children’s Hospital Research Foundation, the National Heart Foundation of Australia, the Australia and New Zealand Children’s Heart Research Centre and Heartkids Australia.