End-stage heart failure and heart transplant in cardiac sarcoidosis: a case series.

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS
European Heart Journal: Case Reports Pub Date : 2024-12-03 eCollection Date: 2024-12-01 DOI:10.1093/ehjcr/ytae635
Maria Francesca Scuppa, Antonella Accietto, Anna Corsini, Maddalena Graziosi, Elena Biagini, Chiara Baldovini, Mario Sabatino, Luciano Potena
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Abstract

Background: Diagnosing cardiac sarcoidosis (CS) is challenging. Immunosuppressive therapies are less effective in end-stage disease, and often heart transplant (HT) is the only available option. We present a series of advanced CS cases, requiring HT, along with a review of the literature evidence in this field.

Case summary: Case 1: a 49-year-old man initially suspected of having arrhythmogenic cardiomyopathy (ACM) presented with heart failure (HF) and recurrent ventricular arrhythmias. The rapid clinical deterioration raised suspicion of an inflammatory aetiology, which was confirmed through endomyocardial biopsy, diagnosing CS. Despite immunosuppressive therapy, HT was required. Case 2: a 36-year-old woman presented with high-grade atrioventricular block and dilated cardiomyopathy (DCM), initially diagnosed as idiopathic. Due to worsening HF, she required HT. The pathological examination of the explanted heart revealed CS. Chronic subclinical antibody-mediated rejection was observed after HT. Case 3: a 44-year-old man presented with syncope and imaging suggesting ACM. He was referred for HT due to high ventricular arrhythmic burden. Cardiac sarcoidosis diagnosis was suspected due to pulmonary involvement and then confirmed on post-explant pathological exam. Post-HT pulmonary and cutaneous sarcoidosis reactivation were observed. Case 4: a 43-year-old man was diagnosed with pulmonary sarcoidosis after lung biopsy. Progression towards DCM was observed despite immunosuppressive therapy. Post-HT was characterized by multiple episodes of graft rejection.

Discussion: This case series provides insights into mid- and long-term outcomes after HT for CS, highlighting the need for careful management of immunosuppression in these patients, balancing the adverse effects of chronic immunosuppression with the prevention of rejection and sarcoidosis recurrence.

心脏结节病终末期心力衰竭和心脏移植:一个病例系列。
背景:诊断心脏结节病(CS)具有挑战性。免疫抑制疗法在终末期疾病中效果较差,通常心脏移植(HT)是唯一可用的选择。我们提出了一系列需要HT的晚期CS病例,并回顾了该领域的文献证据。病例总结:病例1:一名49岁男性,最初怀疑患有心律失常性心肌病(ACM),表现为心力衰竭(HF)和复发性室性心律失常。快速的临床恶化引起了对炎症病因的怀疑,通过心内膜肌活检证实,诊断为CS。尽管免疫抑制治疗,HT是必需的。病例2:一名36岁女性表现为高级别房室传导阻滞和扩张型心肌病(DCM),最初诊断为特发性。由于心衰恶化,她需要HT治疗。移植心脏病理检查示CS。治疗后出现慢性亚临床抗体介导的排斥反应。病例3:44岁男性,表现为晕厥,影像学提示ACM。由于高室性心律失常负荷,他被转诊为HT。由于肺部受累,怀疑心脏结节病的诊断,然后在移植后的病理检查中证实。观察ht后肺部和皮肤结节病的再激活。病例4:一名43岁男性经肺活检诊断为肺结节病。尽管免疫抑制治疗,仍观察到DCM进展。术后以多次移植排斥反应为特征。讨论:本病例系列提供了对CS HT后的中长期结果的见解,强调了对这些患者进行免疫抑制的仔细管理的必要性,平衡慢性免疫抑制与预防排斥反应和结节病复发的不良影响。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
European Heart Journal: Case Reports
European Heart Journal: Case Reports Medicine-Cardiology and Cardiovascular Medicine
CiteScore
1.30
自引率
10.00%
发文量
451
审稿时长
14 weeks
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