Daler Rahimov , Vivian Z. Yan , Danial Ahmad , Nayeem Nasher , Rob Tatum , Moses Im , Eugene Storozynsky , J. Eduardo Rame , Keshava Rajagopal , John W. Entwistle , Howard T. Massey , Vakhtang Tchantchaleishvili
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引用次数: 0
Abstract
Purpose
Patients with systemic amyloidosis with cardiac involvement require careful selection for heart transplantation (HTx) due to the associated poor outcomes. Large databases do not provide sufficient granularity to allow for differentiation between its major subtypes [light-chain (AL) and transthyretin (ATTR) amyloidosis]. We sought to pool the existing data on amyloidosis patients undergoing HTx, and perform stratified analysis based on its major subtypes.
Methods
Electronic search identified adult patients with amyloidosis undergoing HTx. Cohort-level data for 340 patients from 19 studies were extracted and analyzed. Patients were categorized based on amyloid subtype into AL and ATTR groups.
Results
AL amyloidosis was diagnosed at an earlier age compared to ATTR [53 (95 % CI 48; 57) years vs. 63 (55; 71) years, p = 0.03], with greater incidence in the Caucasian population [75 % (60; 87) vs. 39 % (21; 59), p ≤0.01]. Females comprised 33 % (25; 41) of the patients with greater preponderance in AL group [41 % (33; 48) vs. 21 % (8; 36), p = 0.02]. AL patients also had higher involvement of ≥two organs [50 % (29; 70) vs. 15 % (3; 32), p = 0.01]. GI involvement [25 % (6; 50) vs. 0 % (0; 8), p = 0.02], and renal involvement [20 % (8; 34) vs. 0 % (0; 2), p < 0.01] were virtually limited to AL, while ATTR patients had more implantable cardioverter defibrillators placed [64 % (34; 90) vs. 15 % (6; 28), p < 0.01] and trended towards greater incidence of neuropathy [24 % (9; 42) vs. 9 % (2; 19), p = 0.07]. The AL group had a significantly higher incidence of recurrent amyloidosis [16 % (7; 27) vs. 0 % (0; 0), p ≤0.01]. Pooled Kaplan-Meier survival analysis showed worse long-term survival in the AL group (p = 0.02).
Conclusion
Patients with AL amyloidosis showed more widespread systemic involvement and worse long-term survival after HTx compared to patients with ATTR amyloidosis. Protocols for mitigating the recurrence of AL amyloidosis are needed to improve survival in this high-risk subtype.
目的:系统性淀粉样变性合并心脏受累的患者由于预后不良,需要谨慎选择心脏移植。大型数据库没有提供足够的粒度来区分其主要亚型[轻链(AL)和转甲状腺素(ATTR)淀粉样变性]。我们试图汇总现有的淀粉样变性患者接受HTx的数据,并根据其主要亚型进行分层分析。方法对成人淀粉样变性行HTx的患者进行电子搜索。从19项研究中提取并分析了340例患者的队列水平数据。根据淀粉样蛋白亚型将患者分为AL组和ATTR组。结果:与ATTR相比,淀粉样变的诊断年龄更早[53](95% CI 48;57岁vs. 63岁(55;71)岁,p = 0.03],高加索人群发病率更高[75% (60;87) vs. 39% (21;59), p≤0.01]。女性占33% (25;在AL组有较大优势的患者中[41% (33;48) vs. 21% (8;36), p = 0.02]。AL患者也有更高的≥两个器官受累[50% (29;70) vs. 15% (3;32), p = 0.01]。GI受累[25% (6;50) vs. 0% (0;8), p = 0.02],肾脏受累[20% (8;34) vs. 0% (0;2), p <;[0.01]实际上仅限于AL,而ATTR患者放置了更多的植入式心律转复除颤器[64% (34;90) vs. 15% (6;28), p &;[0.01]并倾向于更高的神经病变发生率[24% (9;42) vs. 9% (2;19), p = 0.07]。AL组复发性淀粉样变性的发生率明显高于对照组[16% (7;27) vs. 0% (0;0), p≤0.01]。Kaplan-Meier生存分析显示,AL组的长期生存率较差(p = 0.02)。结论与ATTR淀粉样变患者相比,AL淀粉样变患者HTx术后表现出更广泛的全身累及和更差的长期生存。需要减轻AL淀粉样变复发的方案来提高这种高风险亚型的生存率。
期刊介绍:
Transplantation Reviews contains state-of-the-art review articles on both clinical and experimental transplantation. The journal features invited articles by authorities in immunology, transplantation medicine and surgery.