PDF(Pubmed)
Abstract：
Cardiac amyloidosis (CA) manifests as infiltrative cardiomyopathy with a hypertrophic pattern, usually presenting with heart failure with a preserved ejection fraction. In addition, degenerative valvular heart disease, particularly severe aortic stenosis, is commonly seen in patients with CA. However, amyloid fibril deposition might also infiltrate the conduction system and promote the development of electrical disorders, including ventricular tachyarrhythmias, atrio-ventricular block or acute electromechanical dissociation. These manifestations can increase the risk of sudden cardiac death. This review summarises the pathophysiological mechanisms and risk factors for sudden cardiac death in CA and focuses on the major current concerns regarding medical and device management in this challenging scenario.
摘要：
心脏淀粉样变性（CA）表现为肥大型浸润性心肌病，通常表现为心力衰竭并保留射血分数。此外,退行性心脏瓣膜病,特别是严重的主动脉瓣狭窄，常见于CA患者。然而,淀粉样蛋白原纤维沉积也可能渗入传导系统并促进电紊乱的发展，包括室性心律失常,房室传导阻滞或急性电机械分离。这些表现会增加心源性猝死的风险。这篇综述总结了CA中心脏猝死的病理生理机制和危险因素，并重点介绍了在这种具有挑战性的情况下当前有关医疗和设备管理的主要问题。
