Effect of cibenzoline on biventricular pressure gradients in a pediatric patient with hypertrophic obstructive cardiomyopathy.

This report presents the case of an 8-year-old male with biventricular hypertrophic obstructive cardiomyopathy who achieved a successful reduction of the pressure gradients by treatment with cibenzoline. Ultrasound echocardiography showed marked intraventricular septal hypertrophy and left midventricular and right ventricular outflow obstructions. A cardiac catheterization revealed left midventricular and right outflow obstructions associated with pressure gradients of 100 and 35 mmHg, respectively. An intravenous injection of 28 mg (1.4 mg/kg) cibenzoline immediately improved the biventricular pressure gradients. Therefore, oral cibenzoline treatment was initiated, the dose was increased to 150 mg/day, and both pressure gradients remained low, without any complications at the time of discharge, 4 months later.