Hypertrophic cardiomyopathy in children

Hypertrophic cardiomyopathy is characterized by myocardial hypertrophy that cannot be explained by abnormal loading conditions. Pathological process causes structural, functional and electrophysiological changes of myocardium, leading mainly to diastolic dysfunction, left ventricular outflow tract obstruction (LVOTO) and arrhythmia. Symptoms of heart failure, chest pain, syncope and palpitations account for most of patient symptoms, however, the majority of patients is asymptomatic. Mortality in pediatric population is around 1–2 % per year, mostly due to sudden cardiac death. Therapy is rarely causal. It focuses on pharmacological reduction of symptoms of myocardial dysfunction, if ineffective, patients with LVOTO are referred to surgical mycetoma. Patients with refractory heart failure or arrhythmias are candidates for heart transplantation. Identification of patients with high risk of sudden death, who are candidates for ICD implantation is crucial and can be challenging. Patients with hKMP are discouraged from intense physical activity and competitive sport. (author)