Why the study?
Does cibenzoline reduce biventricular pressure gradients in a patient with biventricular hypertrophic obstructive cardiomyopathy?
Does cibenzoline reduce biventricular pressure gradients in a patient with biventricular hypertrophic obstructive cardiomyopathy?
Cibenzoline successfully reduced biventricular pressure gradients in a young patient with biventricular hypertrophic obstructive cardiomyopathy.
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Hypothesis-generating for cibenzoline in biventricular HOCM; requires prospective trials before clinical adoption.
An 18-year-old male patient with biventricular hypertrophic obstructive cardiomyopathy (HOCM) had successful reduction of the pressure gradients by cibenzoline. At 11 months after birth, he was first diagnosed with cardiac murmurs and by the age of 5 years, he was diagnosed with subpulmonic infundibular stenosis with a pressure gradient of 10 mmHg by cardiac catheterization. At the age of 14, re-catherterization revealed hypertrophic cardiomyopathy with isolated obstruction of the right ventricular outflow tract, with a pressure gradient of 70 mmHg, but no obstruction in the left ventricle. He began daily treatment with 30 mg propranolol. At the age of 18, he was admitted for cardiac evaluation. An echocardiogram revealed left mid-ventricular and subpulmonic obstructions associated with pressure gradients of 88 mmHg and 65 mmHg, respectively. A single oral dose of 200 mg of cibenzoline decreased the pressure gradients in the left and right ventricles (38 mmHg and 36 mmHg, respectively). He was then given 300 mg daily of cibenzoline, and both pressure gradients remained low without any complications 8 months later at the time of discharge.
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Hiasa et al. (2002) studied this question.
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