Hypertrophic cardiomyopathy associated with mitochondrial disease in children / 中国实用儿科杂志

ABSTRACT

Mitochondrial disease is a heterogeneous group of hereditary diseases caused by the defects in the mitochondrial respiratory chain and abnormal cellular energy metabolism.Heart is one of the most common organs involved,and hypertrophic cardiomyopathy is the most common and important type of cardiac involvement in mitochondrial disease. Hypertrophic cardiomyopathy in the patients with mitochondrial disease with childhood onset is more common than those with adulthood onset. Mortality in children with cardiac involvement caused by mitochondrial disease is significantly higher than that in children without cardiac involvement,so the early diagnosis and treatment is very important. But the early diagnosis is still difficult due to the complexity of clinical manifestations of mitochondrial disease. There is no specific treatment for mitochondrial disease and its associated hypertrophic cardiomyopathy,so supportive therapy is still the main treatment.