Herrador Lorena, Gayán-Ordás Jara, Llatjós Roger, Claver-Garrido Eduard, González-Costello José
Department of Cardiology, Hospital Universitari de Bellvitge, C/Feixa Llarga s/n, 08907, L'Hospitalet de Llobregat, Barcelona, Spain.
Bioheart-Cardiovascular Diseases Group, Cardiovascular, Respiratory and Systemic Diseases and Cellular Aging Program, Institut d'Investigació Biomèdica de Bellvitge-IDIBELL, C/Feixa Llarga s/n, L'Hospitalet de Llobregat, 08907 Barcelona, Spain.
Eur Heart J Case Rep. 2025 Apr 2;9(5):ytaf149. doi: 10.1093/ehjcr/ytaf149. eCollection 2025 May.
Endomyocardial fibrosis (EMF) is a challenging disease that leads to severe heart failure (HF) due to progressive fibrosis. Diet, parasitic infections, autoimmune disorders, and genetic predisposition have been advocated in EMF pathogenesis, and treatment options for EMF are limited with scarce evidence supporting heart transplantation (HTx).
A 38-year-old man was diagnosed with EMF with biventricular involvement. The diagnostic work-up ruled out eosinophilia, infections, and autoimmune conditions. The patient rapidly deteriorated, leading to cardiogenic shock with multiorgan failure, and an emergency HTx was performed. Three months later, the graft developed biventricular hypertrophy with atrial fibrosis and the endomyocardial biopsy (EMB) showed extensive inflammation and myocardial damage, compatible with Grade 2R (G3a) cellular rejection. After steroid pulses, the follow-up EMB reveals subendocardial fibrosis and microcalcifications, suggesting the possibility of an EMF recurrence. Nevertheless, the patient had a good clinical outcome, remaining asymptomatic with good graft function 2 years after the transplant.
This is the first reported case of suspected EMF recurrence following HTx. Given the unknown pathogenesis of EMF, close monitoring is crucial, though HTx appears to be a viable and successful treatment option for these patients.
心内膜心肌纤维化（EMF）是一种具有挑战性的疾病，由于进行性纤维化可导致严重心力衰竭（HF）。饮食、寄生虫感染、自身免疫性疾病和遗传易感性在EMF发病机制中被提及，EMF的治疗选择有限，支持心脏移植（HTx）的证据稀少。
一名38岁男性被诊断为双心室受累的EMF。诊断性检查排除了嗜酸性粒细胞增多、感染和自身免疫性疾病。患者病情迅速恶化，导致心源性休克伴多器官功能衰竭，遂进行了紧急HTx。三个月后，移植心脏出现双心室肥厚伴心房纤维化，心内膜心肌活检（EMB）显示广泛炎症和心肌损伤，符合2R级（G3a）细胞排斥反应。类固醇冲击治疗后，随访EMB显示心内膜下纤维化和微钙化，提示EMF复发的可能性。尽管如此，患者临床结局良好，移植后2年仍无症状，移植心脏功能良好。
这是首例报道的HTx后疑似EMF复发的病例。鉴于EMF发病机制不明，密切监测至关重要，尽管HTx似乎是这些患者可行且成功的治疗选择。
