Hamada Mareomi, Ikeda Shuntaro, Shigematsu Yuji
Division of Cardiology, Uwajima City Hospital, 1-1 Goten-machi, Uwajima, Ehime 798-8510, Japan.
Division of Cardiology, Uwajima City Hospital, 1-1 Goten-machi, Uwajima, Ehime 798-8510, Japan.
J Cardiol. 2014 Jul;64(1):1-10. doi: 10.1016/j.jjcc.2014.02.022. Epub 2014 Apr 13.
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.
我们回顾了肥厚型心肌病（HCM）患者的自然病史。还评估了药物治疗对自然病史、左心室（LV）功能和左心室重塑的影响。心源性猝死和终末期心力衰竭是HCM最严重的并发症。年龄<30岁和有早发猝死家族史是HCM患者心源性猝死的危险因素。终末期心力衰竭并非HCM患者特有的额外现象，而是大多数此类患者疾病的自然病程。心力衰竭发生后，进展至心源性死亡非常迅速。诊断时年龄较小、有HCM家族史以及更大的室壁厚度与发生终末期心力衰竭的可能性更大相关。β受体阻滞剂和钙拮抗剂均不能预防这种转变。Ia类抗心律失常药物双异丙吡胺和西苯唑啉可用于降低左心室压力梯度。与双异丙吡胺不同，西苯唑啉几乎没有抗胆碱能活性；因此，该药易于适应长期使用。除降低左心室压力梯度外，西苯唑啉还可改善HCM患者的左心室舒张功能障碍，并诱导左心室肥厚消退。西苯唑啉治疗相关的通过激活Na+/Ca2+交换体导致细胞内Ca2+浓度降低，可能与HCM相关病症的改善密切相关。西苯唑啉有可能预防典型HCM进展至终末期心力衰竭。
