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Sudden cardiac death risk in pediatric cardiomyopathy may vary by phenotype
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In children with cardiomyopathy, the likelihood of sudden cardiac death may be predicted by cardiomyopathy phenotype, family history, severity of systolic dysfunction and degree of left ventricular hypertrophy, researchers reported in the Journal of the American College of Cardiology.
Researchers evaluated the incidence and risk factor for sudden cardiac death in 289 children younger than 10 years enrolled in the National Australian Childhood Cardiomyopathy Study (NACCS). The NACCS is an ongoing, longitudinal study assessing all children in Australia diagnosed with cardiomyopathy from 1987 to 1996. Each patient was categorized based on WHO classification: dilated cardiomyopathy, hypertrophic cardiomyopathy, restrictive cardiomyopathy or LV noncompaction.
During a median follow-up of 12 years, sudden cardiac death occurred in 5.5% of children. Risk for sudden cardiac death varied based on cardiomyopathy phenotype (P = .007). At 15 years, the cumulative incidence of sudden cardiac death was 23% among patients with LV noncompaction, 12% among patients with restrictive cardiomyopathy, 6% among patients with hypertrophic cardiomyopathy and 5% among patients with dilated cardiomyopathy, according to the results.
Risk factors for sudden cardiac death in this population included older age at diagnosis, positive family history of cardiomyopathy and severity of LV dysfunction. “A higher posterior wall thickness z score was the sole risk factor identified for patients with hypertrophic cardiomyopathy,” the researchers wrote.
Larger childhood studies of individual cardiomyopathy phenotypes may be helpful for the identification of children at the highest risk for sudden cardiac death.
“Continuing follow-up of this cohort into adulthood is likely to reveal an ongoing risk of sudden cardiac death in this population,” the researchers wrote. – by Jennifer Byrne
Disclosure: This study was supported by the Royal Children’s Hospital Research Foundation, the National Heart Foundation of Australia, the Australia and New Zealand Children’s Heart Research Center and Heartkids Australia. One researcher reports advising for Actelion Pharmaceuticals.
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