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      Advances in medical treatment of hypertrophic cardiomyopathy.

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          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.

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          Author and article information

          Journal
          J Cardiol
          Journal of cardiology
          Elsevier BV
          1876-4738
          0914-5087
          Jul 2014
          : 64
          : 1
          Affiliations
          [1 ] Division of Cardiology, Uwajima City Hospital, 1-1 Goten-machi, Uwajima, Ehime 798-8510, Japan. Electronic address: mhamada@uwajima-mh.jp.
          [2 ] Division of Cardiology, Uwajima City Hospital, 1-1 Goten-machi, Uwajima, Ehime 798-8510, Japan.
          [3 ] Clinical Nursing, Ehime University Graduate School of Medicine, Shitsukawa, Toon-City, Ehime 791-0295, Japan.
          Article
          S0914-5087(14)00077-X
          10.1016/j.jjcc.2014.02.022
          24735741
          5824e002-bfbf-4a10-abfc-ab24efb3c06d
          History

          Beta-blocker,Calcium antagonist,Cibenzoline,Disopyramide,Hypertrophic cardiomyopathy

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