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Japanese-variant or apical hypertrophic cardiomyopathy (HCM) is a specific type of HCM, first described in Japan and initially thought to carry a benign prognosis. However, current evidence suggests that these patients experience severe symptoms and are at increased risk of ventricular arrhythmias and death, especially in the presence of an apical akinetic chamber. The management of patients who do not respond to medical therapy is challenging. We describe a patient with Japanese-variant HCM, with an apical akinetic chamber and severe symptoms who failed medical therapy. The use of dual chamber pacing relieved obstruction and significantly improved the patient's symptoms.

Dual Chamber Pacing Relieves Obstruction in Japanese-Variant Hypertrophic Cardiomyopathy