Scuppa Maria Francesca, Accietto Antonella, Corsini Anna, Graziosi Maddalena, Biagini Elena, Baldovini Chiara, Sabatino Mario, Potena Luciano
Cardiology Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Policlinico Sant'Orsola Malpighi, Via Massarenti 9, 40138 Bologna, Italy.
Department of Medical and Surgical Sciences, University of Bologna, Via Massarenti 9, 40138 Bologna, Italy.
Eur Heart J Case Rep. 2024 Dec 3;8(12):ytae635. doi: 10.1093/ehjcr/ytae635. eCollection 2024 Dec.
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 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.
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.
诊断心脏结节病（CS）具有挑战性。免疫抑制疗法在疾病终末期效果较差，心脏移植（HT）往往是唯一可行的选择。我们报告了一系列需要进行心脏移植的晚期CS病例，并对该领域的文献证据进行了综述。
病例1：一名49岁男性最初被怀疑患有致心律失常性心肌病（ACM），出现心力衰竭（HF）和反复发作的室性心律失常。临床迅速恶化引发了对炎症病因的怀疑，经心内膜活检确诊为CS。尽管进行了免疫抑制治疗，仍需要进行心脏移植。病例2：一名36岁女性出现高度房室传导阻滞和扩张型心肌病（DCM），最初诊断为特发性。由于心力衰竭恶化，她需要进行心脏移植。移植心脏的病理检查显示为CS。心脏移植后观察到慢性亚临床抗体介导的排斥反应。病例3：一名44岁男性出现晕厥，影像学检查提示ACM。由于室性心律失常负担高，他被转诊进行心脏移植。由于肺部受累，怀疑为心脏结节病，移植后病理检查确诊。心脏移植后观察到肺部和皮肤结节病复发。病例4：一名43岁男性经肺活检诊断为肺结节病。尽管进行了免疫抑制治疗，但仍观察到病情进展为DCM。心脏移植后表现为多次移植排斥反应。
本病例系列提供了关于CS患者心脏移植中长期结局的见解，强调了对这些患者进行免疫抑制仔细管理的必要性，平衡慢性免疫抑制的不良反应与预防排斥反应和结节病复发。
