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心脏结节病所致终末期心力衰竭的心脏移植

Heart transplantation for end-stage heart failure due to cardiac sarcoidosis.

作者信息

Perkel D, Czer L S C, Morrissey R P, Ruzza A, Rafiei M, Awad M, Patel J, Kobashigawa J A

机构信息

Division of Cardiology, Cedars-Sinai Heart Institute, Los Angeles, California, USA.

出版信息

Transplant Proc. 2013 Jul-Aug;45(6):2384-6. doi: 10.1016/j.transproceed.2013.02.116.

DOI:10.1016/j.transproceed.2013.02.116
PMID:23953552
Abstract

BACKGROUND

Cardiac sarcoidosis with end-stage heart failure has a poor prognosis without transplantation. The rates of sarcoid recurrence and rejection are not well established after heart transplantation.

METHODS

A total of 19 heart transplant recipients with sarcoid of the explanted heart were compared with a contemporaneous control group of 1,050 heart transplant recipients without cardiac sarcoidosis. Assessed outcomes included 1st-year freedom from any treated rejection, 5-year actuarial survival, 5-year freedom from cardiac allograft vasculopathy (CAV), 5-year freedom from nonfatal major adverse cardiac events (NF-MACE), and recurrence of sarcoid in the allograft or other organs. Patients with sarcoidosis were maintained on low-dose corticosteroids after transplantation.

RESULTS

There were no significant differences between the sarcoid and control groups in 1st-year freedom from any treated rejection (79% and 90%), 5-year posttransplantation survival (79% and 83%), 5-year freedom from CAV (68% and 78%), and 5-year freedom from NF-MACE (90% and 88%). Causes of death (n = 5) in the sarcoid group were coccidioidomycosis, pneumonia, rejection, hemorrhage, and CAV. No patient had recurrence of sarcoidosis in the cardiac allograft. Three of 19 patients (16%) experienced recurrence of extracardiac sarcoid, with no mortality.

CONCLUSIONS

Patients with cardiac sarcoidosis undergoing heart transplantation have acceptable long-term outcomes without evidence of recurrence of sarcoidosis in the allograft when maintained on low-dose corticosteroids. Progression of extracardiac sarcoid was uncommon, possibly related to immunosuppression. In patients with cardiac sarcoidosis, heart transplantation is a viable treatment modality.

摘要

背景

患有终末期心力衰竭的心脏结节病患者若不进行移植,预后较差。心脏移植后结节病复发和排斥反应的发生率尚未明确。

方法

将19例接受心脏移植且移植心脏患有结节病的患者与同期1050例未患心脏结节病的心脏移植受者组成的对照组进行比较。评估的结果包括1年无任何治疗性排斥反应、5年预期生存率、5年无心脏移植血管病变(CAV)、5年无非致命性重大不良心脏事件(NF-MACE)以及移植心脏或其他器官中结节病的复发情况。结节病患者移植后维持低剂量皮质类固醇治疗。

结果

结节病组和对照组在1年无任何治疗性排斥反应(79%和90%)、移植后5年生存率(79%和83%)、5年无CAV(68%和78%)以及5年无NF-MACE(90%和88%)方面无显著差异。结节病组死亡原因(n = 5)为球孢子菌病、肺炎、排斥反应、出血和CAV。心脏移植中无患者出现结节病复发。19例患者中有3例(16%)发生心脏外结节病复发,无死亡病例。

结论

接受心脏移植的心脏结节病患者在维持低剂量皮质类固醇治疗时,长期预后良好,且移植心脏无结节病复发迹象。心脏外结节病进展不常见,可能与免疫抑制有关。对于心脏结节病患者,心脏移植是一种可行的治疗方式。

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