Key result
Extended septal myectomy in symptomatic pediatric patients with obstructive hypertrophic cardiomyopathy provides excellent relief of symptoms and improves late survivorship compared to the natural history of the disease.
Septal myectomy is a safe and effective surgical intervention for children with obstructive hypertrophic cardiomyopathy who remain symptomatic despite medical therapy, providing excellent long-term survival.
Hypertrophic cardiomyopathy (HCM) occurs in 1 of 500 adults and is considered to be one of the most common causes of death in young people under 35 years of age. Children with HCM are usually asymptomatic and the overall annual mortality beyond the first year of life is 1%. Septal myectomy is safe and effective in children with obstructive HCM and published data shows improved late survival compared to untreated HCM. Patient selection and surgical expertise remain critical components to ensuring successful outcomes of septal myectomy, particularly when considering prophylactic myectomy in a seemingly asymptomatic patient.
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Arghami et al. (2017) conducted a review in Hypertrophic cardiomyopathy. Septal myectomy vs. Medical therapy or natural history was evaluated. Extended septal myectomy in symptomatic pediatric patients with obstructive hypertrophic cardiomyopathy provides excellent relief of symptoms and improves late survivorship compared to the natural history of the disease.
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