Angsubhakorn Natthapon, Agdamag Arianne, Sumransub Nuttavut, Velangi Pratik, Freund Robert, Martin Cindy M, Alexy Tamas
Department of Medicine, University of Minnesota, Minneapolis, MN, USA.
Division of Cardiology, Department of Medicine, University of Minnesota, Minneapolis, MN, USA.
Respir Med Case Rep. 2021 Jan 24;32:101349. doi: 10.1016/j.rmcr.2021.101349. eCollection 2021.
A 66-year-old male with recent diagnosis of heart failure with reduced ejection fraction was referred to our institution for management of cardiogenic/vasodilatory shock. During his evaluation, he suffered a sudden cardiac arrest from refractory ventricular tachycardia/fibrillation (VT/VF) despite normal electrolytes and no evidence of prior ventricular arrhythmias. He was placed on rescue peripheral veno-arterial extracorporeal membrane oxygenation support (VA-ECMO) for 4 days and was decannulated without end-organ damage. Continued workup revealed Mayo stage IV immunoglobulin light chain (AL) amyloidosis. Unfortunately, he developed acute cerebellar hemorrhage several days later. Autopsy findings were consistent with AL amyloidosis, with extensive cardiac fibrosis and amyloid deposition in the myocardium and vasculature. While the most common cause of cardiac death in patients with amyloidosis is severe bradycardia and pulseless electrical activity, sustained ventricular arrhythmias have been reported. The use of implantable cardioverter defibrillators (ICD) is highly debated in this population given the lack of survival benefit. Our patient also developed refractory VT/VF arrest, and ICD shocks would not have rescued him while causing significant distress. Emergent VA-ECMO cannulation allowed us to make a diagnosis, yet this intervention cannot be routinely recommended given the limited survival of patients with AL amyloidosis.
一名66岁男性，近期被诊断为射血分数降低的心力衰竭，因心源性/血管扩张性休克被转诊至我院治疗。在评估过程中，尽管电解质正常且既往无室性心律失常证据，但他仍因难治性室性心动过速/心室颤动（VT/VF）发生心脏骤停。他接受了4天的抢救性外周静脉-动脉体外膜肺氧合支持（VA-ECMO），撤管时未出现终末器官损伤。进一步检查发现为梅奥IV期免疫球蛋白轻链（AL）淀粉样变性。不幸的是，几天后他发生了急性小脑出血。尸检结果与AL淀粉样变性相符，心肌和脉管系统存在广泛的心脏纤维化和淀粉样沉积。虽然淀粉样变性患者心脏死亡的最常见原因是严重心动过缓和无脉电活动，但也有持续性室性心律失常的报道。鉴于缺乏生存获益，在这一人群中植入式心律转复除颤器（ICD）的使用存在很大争议。我们的患者也发生了难治性VT/VF心脏骤停，ICD电击不仅无法挽救他的生命，还会给他带来极大痛苦。紧急VA-ECMO插管使我们得以做出诊断，但鉴于AL淀粉样变性患者的有限生存期，不能常规推荐这种干预措施。
