Hamudi Jihad, Karkabi Basheer, Zisman Devy, Shiran Avinoam
Department of Cardiovascular Medicine, Lady Davis Carmel Medical Center, 7 Michal Street, Haifa 34362, Israel.
The Ruth and Bruce Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa 3200003, Israel.
Eur Heart J Case Rep. 2020 Nov 5;4(6):1-5. doi: 10.1093/ehjcr/ytaa417. eCollection 2020 Dec.
Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a rare multisystem disease characterized by asthma, rhinosinusitis, and eosinophilia. Cardiac involvement, present in half the patients, may be life threatening.
A young woman with long-standing asthma and nasal polyposis was admitted with new-onset dyspnoea, sinus tachycardia, and eosinophilia. She had severe biventricular thrombosis and severe tricuspid regurgitation (TR) on echocardiography, with preserved ejection fraction of both ventricles. Cardiac magnetic resonance (CMR) imaging showed diffuse subendocardial late gadolinium enhancement (LGE). She had a positive test for perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) confirming the diagnosis of ANCA positive EGPA. She was treated with anticoagulation, high-dose corticosteroids, cyclophosphamide, and rituximab with gradual resolution of her symptoms. Follow-up echocardiography showed significant improvement in ventricular thrombi and TR but could not reliably exclude residual ventricular thrombus. Repeat CMR at 11 months confirmed complete resolution of both ventricular thrombi and near complete resolution of LGE.
Cardiac involvement in EGPA, a rare cause of heart failure, can manifest as severe biventricular thrombosis and severe TR, resulting in heart failure with preserved ejection fraction. Combined immunosuppression and anticoagulation can lead to complete remission within a year. CMR is instrumental for both diagnosis and follow-up of EGPA, allowing for safe discontinuation of oral anticoagulation.
嗜酸性肉芽肿性多血管炎（EGPA），既往称为变应性肉芽肿性血管炎，是一种罕见的多系统疾病，其特征为哮喘、鼻窦炎和嗜酸性粒细胞增多。半数患者存在心脏受累，可能危及生命。
一名患有长期哮喘和鼻息肉病的年轻女性因新发呼吸困难、窦性心动过速和嗜酸性粒细胞增多入院。超声心动图显示她有严重的双心室血栓形成和严重的三尖瓣反流（TR），双心室射血分数保留。心脏磁共振（CMR）成像显示弥漫性心内膜下延迟钆增强（LGE）。她的核周抗中性粒细胞胞浆抗体（p-ANCA）检测呈阳性，确诊为ANCA阳性EGPA。她接受了抗凝、大剂量皮质类固醇、环磷酰胺和利妥昔单抗治疗，症状逐渐缓解。随访超声心动图显示心室血栓和TR有显著改善，但不能可靠地排除残留心室血栓。11个月时重复CMR证实心室血栓完全消失，LGE几乎完全消失。
EGPA导致的心脏受累是心力衰竭的罕见原因，可表现为严重的双心室血栓形成和严重的TR，导致射血分数保留的心力衰竭。联合免疫抑制和抗凝可在一年内导致完全缓解。CMR对EGPA的诊断和随访都有帮助，可安全停用口服抗凝药。
