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Updated: May 11, 2026

Implantation of Total Artificial Heart in Congenital Heart Disease
Published on: July 18, 2014
肥大型閉塞性心筋症におけるマバカムテン:肝腎併存疾患を有する症例報告
Liping Ma1, Fengqin Li2, Sophia Dong3
1Department of Cardiovascular Medicine, Shulan (Hangzhou) Hospital, Shulan International Medical College, Zhejiang Shuren University, Hangzhou, P. R. China.
Rationale:
Mavacamten is an innovative drug for hypertrophic obstructive cardiomyopathy (HOCM). It improves cardiac function by reducing left ventricular outflow tract obstruction. However, safety data in patients with liver and kidney impairment are limited.
Patient Concerns:
A 68-year-old man was admitted with a history of HOCM, heart failure, gout, and chronic kidney and liver impairment. He was admitted because of worsening heart failure symptoms and an episode of upper gastrointestinal bleeding. The combined burden of these illnesses significantly impaired his ability to perform daily activities independently, leading to hospitalization for optimized management of his advanced cardiac disease.
Diagnoses:
Hypertrophic obstructive cardiomyopathy (apical aneurysm, post-cardiac resynchronization therapy), New York Heart Association class III. Hepatic schistosomiasis with portal hypertension, splenomegaly, upper GI bleeding, moderate anemia. Chronic gout with tophaceous deposits. Chronic kidney disease stage IIIb.
Interventions:
Initiated mavacamten 2.5 mg once daily for severe left ventricular outflow tract obstruction refractory to maximally tolerated beta-blockers.
Outcomes:
The patient showed significant improvement after treatment. The New York Heart Association class decreased from III to I-II, and the LVOTG dropped from 105 to 16 mm Hg. Meanwhile, liver and kidney functions remained stable during the course of treatment.
Lessons:
This case highlights the complexity of managing HOCM with multiorgan comorbidities, emphasizing the role of novel therapies (e.g., mavacamten) in refractory outflow obstruction and the need for tailored multidisciplinary approaches.
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関連する概念動画
Cardiomyopathy I: Introduction and Classification
Cardiomyopathy II: Dilated Cardiomyopathy
Cardiomyopathy III: Hypertrophic Cardiomyopathy
Cardiomyopathy IV: Restrictive Cardiomyopathy
Cardiomyopathy V: Interprofessional Care
Hypertension III: Clinical Manifestations and Diagnostic Studies