title: Advances in medical treatment of hypertrophic cardiomyopathy.
abstract: We reviewed the natural history of patients with hypertrophic cardiomyopathy (HCM). The effect of medical treatments on natural history, left ventricular (LV) functions and LV remodeling was also evaluated. Sudden cardiac death and end-stage heart failure are the most serious complications of HCM. Age <30 years and a family history of sudden premature death are risk factors for sudden cardiac death in HCM patients. End-stage heart failure is not a specific additional phenomenon observed in patients with HCM, but is the natural course of the disease in most of those patients. After the occurrence of heart failure, the progression to cardiac death is very rapid. Young age at diagnosis, a family history of HCM, and greater wall thickness are associated with a greater likelihood of developing end-stage heart failure. Neither beta-blockers nor calcium antagonists can prevent this transition. The class Ia antiarrhythmic drugs, disopyramide and cibenzoline are useful for the reduction of LV pressure gradient. Unlike disopyramide, cibenzoline has little anticholinergic activity; therefore, this drug can be easily adapted to long-term use. In addition to the reduction in LV pressure gradient, cibenzoline can improve LV diastolic dysfunction, and induce regression of LV hypertrophy in patients with HCM. A decrease in intracellular Ca(2+) concentration through the activation of the Na(+)/Ca(2+) exchanger associated with cibenzoline therapy is likely to be closely related with the improvement in HCM-related disorders. It is possible that cibenzoline can prevent the progression from typical HCM to end-stage heart failure.
authors.affiliation: Division of Cardiology, Uwajima City Hospital, 1-1 Goten-machi, Uwajima, Ehime 798-8510, Japan. Electronic address: mhamada@uwajima-mh.jp.
authors.forename: Mareomi
authors.initials: M
authors.lastname: Hamada
authors.affiliation: Division of Cardiology, Uwajima City Hospital, 1-1 Goten-machi, Uwajima, Ehime 798-8510, Japan.
authors.forename: Shuntaro
authors.initials: S
authors.lastname: Ikeda
authors.affiliation: Clinical Nursing, Ehime University Graduate School of Medicine, Shitsukawa, Toon-City, Ehime 791-0295, Japan.
authors.forename: Yuji
authors.initials: Y
authors.lastname: Shigematsu
mesh_terms: D000293:Adolescent; D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D000889:Anti-Arrhythmia Agents / Q000627:therapeutic use; D002121:Calcium Channel Blockers / Q000627:therapeutic use; D024741:Cardiomyopathy, Hypertrophic, Familial / Q000150:complications / Q000188:drug therapy*; D016757:Death, Sudden, Cardiac / Q000209:etiology / Q000517:prevention & control; D003971:Diastole; D018450:Disease Progression; D004206:Disopyramide / Q000627:therapeutic use; D005260:Female; D006333:Heart Failure / Q000209:etiology / Q000517:prevention & control; D006801:Humans; D007093:Imidazoles / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D013599:Systole; D018487:Ventricular Dysfunction, Left / Q000188:drug therapy / Q000209:etiology; D020257:Ventricular Remodeling
chemical_list: D000319:Adrenergic beta-Antagonists; D000889:Anti-Arrhythmia Agents; D002121:Calcium Channel Blockers; D007093:Imidazoles; D004206:Disopyramide; C032151:cifenline
keywords: Beta-blocker; Calcium antagonist; Cibenzoline; Disopyramide; Hypertrophic cardiomyopathy