JCE：心肌淀粉样变性患者的ICD治疗

心肌淀粉样变性主要表现为心室肌及瓣膜弥漫性、对称性肥厚，患者多出现限制性心肌病表现，该病的预后不佳，死亡率较高，主要死亡原因为心力衰竭和恶性心律失常。近年来，植入式转复除颤器（ICD）越来越多的用于心脏性猝死的一级预防或二级预防，许多心肌淀粉样变性的患者进行了ICD治疗，然而目前关于心肌淀粉样变的患者能否从ICD中获益还不清楚，目前还缺乏大规模临床研究报道，据此Lin G等进行了一项临床研究，近期发表在JCE杂志，该研究通过长期随访探讨心肌淀粉样变性的患者ICD疗效。
该研究共入选53例心肌淀粉样变性患者，均来自2000年至2009年于Mayo临床医学中心住院的患者，其中33例患者为原发性淀粉样变（AL），10例为老年性淀粉样变，9例为家族性淀粉样变，1例为继发性淀粉样变（AA）。所有患者均植入了ICD，其中用于心脏性猝死一级预防的患者41例，占77%，用于二级预防的患者12例，占23%。随访1年后，32%的患者出现了正确放电，几乎均发生在AL患者，共15例患者发生正确放电，12例为AL，2例为老年性淀粉样变，1例为AA。既往存在心脏骤停或持续性室速的患者ICD放电率较高，左室射血分数低及存在晕厥与ICD放电无明显相关性。
通过该项研究可得出以下结论：心肌淀粉样变性的患者ICD植入后正确放电率较高，尤其是AL患者，因此ICD可有效预防心脏淀粉样变性患者心脏性猝死的发生，然而正确的ICD放电并不能够改善患者的生存率，心肌淀粉样变性的患者预后不佳，因此我们在选择患者时因充分考虑，权衡利弊。

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Implantable Cardioverter Defibrillators in Patients with Cardiac Amyloidosis.
BACKGROUND
Cardiac amyloidosis (CA) is associated with increased risk of sudden cardiac arrest. Although ICD therapy improves survival in patients with cardiomyopathy due to other etiologies, the benefit of ICD therapy in patients with CA is unclear in large part due to limited data on the precise mechanism of sudden cardiac arrest and selection of patients with cardiac amyloidosis for ICD therapy.
OBJECTIVE
The objective was to determine the benefit of ICD therapy in cardiac amyloidosis.
METHODS
We reviewed all ICD implant indications, procedures, and therapies, of CA patients evaluated at Mayo Clinic between 2000 and 2009.
RESULTS
A total of 53 patients with CA (33 AL, 10 senile, 9 familial, and 1 AA) who underwent ICD implantation were included. Indication for ICD implantation was for primary prevention of sudden cardiac arrest in 41 (77%) patients and secondary prevention in 12 (23%) patients. The rate of appropriate ICD shocks was 32% in the first year and was observed almost exclusively in AL amyloidosis patients, occurring in 15 patients (12 AL amyloidosis, 2 senile, 1 AA). Appropriate ICD shocks were more frequent in patients with prior sudden cardiac arrest or sustained ventricular arrhythmias (secondary prevention indication), and less frequent in patients who presented with decreased ejection fraction or syncope.
CONCLUSIONS
A high rate of appropriate ICD shocks was observed especially in patients with AL-type amyloidosis. However, appropriate ICD therapy did not translate into overall survival benefit, suggesting that selection of patients with CA who might be candidates for ICD is imprecise.