Article Details

There has not been an evidence-based medication established for pediatric hypertrophic obstructive cardiomyopathy. We report the clinical details and treatment of a 14-year-old boy with heart failure caused by hypertrophic obstructive cardiomyopathy, which was first diagnosed when he was an infant. He complained of breathlessness while climbing stairs and chest pain. Cibenzoline, 450mg, and metoprolol, 60mg, were administered. Although his symptoms disappeared, the left ventricular outflow tract obstruction did not improve. He had a MYH7 (β-Cardiac Myosin Heavy Chain) mutation [c.2609G>T (p.Arg 870Leu)] and an interventricular wall thickness of more than 30 mm. When he was 17 years old, amiodarone, 100mg, was started for the prevention of ventricular arrhythmia and sudden death. After 7 years, the maximum peak flow at the left ventricular outflow tract decreased from 5.1m/s to 1.5m/s, and his brain natriuretic peptide level went from 310 pg/ml to 8 pg/ml. The New York Heart Association class improved from the second to the first grade. There were no side effects for each medication. The pressure gradient in the left ventricular outflow tract and heart failure in this patient with hypertrophic obstructive cardiomyopathy improved with combination therapy including amiodarone.

Keywords: Hypertrophic obstructive cardiomyopathy; Heart failure; MYH7 (β-cardiac myosin heavy chain); Amiodarone; Cibenzoline