Dual chamber pacing relieves obstruction in Japanese-variant hypertrophic cardiomyopathy

Area of Science:

• Cardiology
• Cardiovascular Medicine

Background:

• Japanese-variant hypertrophic cardiomyopathy (HCM), or apical HCM, was initially considered benign.
• Recent evidence indicates severe symptoms and increased risk of arrhythmias and death in these patients, particularly with an apical akinetic chamber.
• Management of medically refractory HCM patients presents significant challenges.

Purpose of the Study:

• To present a case of Japanese-variant HCM with an apical akinetic chamber and severe symptoms refractory to medical treatment.
• To evaluate the efficacy of dual chamber pacing in managing such a complex case.

Main Methods:

• Case report of a patient with Japanese-variant HCM, apical akinetic chamber, and severe symptoms.
• Patient failed to respond to conventional medical therapy.
• Intervention involved the use of dual chamber pacing.

Main Results:

• Dual chamber pacing successfully relieved left ventricular outflow tract obstruction.
• Significant improvement in the patient's severe symptoms was observed post-pacing.
• The intervention demonstrated a positive impact on symptom management in a challenging HCM subtype.

Conclusions:

• Dual chamber pacing is a viable therapeutic option for patients with Japanese-variant HCM and an apical akinetic chamber who are refractory to medical therapy.
• This approach can effectively alleviate obstruction and improve clinical outcomes.
• Further research may explore the broader application of pacing in managing obstructive HCM variants.