Ono Ryohei, Iwahana Togo, Kato Hirotoshi, Okada Sho, Kobayashi Yoshio
Department of Cardiovascular Medicine, Chiba University Graduate School of Medicine, 1-8-1 Inohana, Chuo-ku, Chiba 260-8670, Japan.
Int J Cardiol Heart Vasc. 2021 Nov 25;37:100915. doi: 10.1016/j.ijcha.2021.100915. eCollection 2021 Dec.
Hypereosinophilic syndrome (HES) is defined by persistently elevated blood eosinophil levels and is associated with evidence of organ damage. Cardiovascular involvement in HES is most commonly associated with Loffler endocarditis (cardiac HES). Cardiac HES is typically characterized by progressive subendocardial fibrosis with overlying mural thrombus formation, leading to restrictive dysfunction of the left ventricle. The thrombus from cardiac HES could result in cardiogenic stroke; however, most of the stroke cases with HES were not associated with huge thromboembolism rather multiple infarcts in the watershed area. The major clinical features of 97 previously reported cases of stroke with HES are as follows: the median age was 52 years, of which 61 (63%) were men; the initial presenting symptoms were neurological (73%), followed by headache (16%), respiratory symptoms (9%), and visual symptoms (9%). Almost half of the cases were diagnosed with cardiac HES. The characteristics of cardiac findings were mural thrombi, endomyocardial fibrosis, and a restrictive pattern of heart failure. Cerebral findings revealed 78 cases (80%) were described as multiple infarctions and 55 cases (57 %) were involved with watershed areas, whereas 11 cases (11%) were described as embolic stroke for one proximal large-vessel occlusion. Regarding treatment, 71 (73%), 28 (29%), and 16 (16%) patients were treated with steroids, anticoagulants, and antiplatelets, respectively. The overall mortality and recovery rates were 11% and 89%, respectively. Physicians should know most cases of stroke with HES are characterized by multiple infarctions in the watershed area, and cardiac HES is not always associated with stroke.
高嗜酸性粒细胞综合征(HES)的定义是血液嗜酸性粒细胞水平持续升高,并伴有器官损害的证据。HES的心血管受累最常与吕弗勒心内膜炎(心脏HES)相关。心脏HES的典型特征是进行性心内膜下纤维化并伴有附壁血栓形成,导致左心室限制性功能障碍。心脏HES形成的血栓可导致心源性卒中;然而,大多数HES相关的卒中病例并非与巨大血栓栓塞有关,而是与分水岭区域的多发性梗死有关。先前报道的97例HES相关卒中病例的主要临床特征如下:中位年龄为52岁,其中61例(63%)为男性;初始症状以神经系统症状为主(73%),其次是头痛(16%)、呼吸道症状(9%)和视觉症状(9%)。几乎一半的病例被诊断为心脏HES。心脏检查结果的特征是附壁血栓、心内膜心肌纤维化和心力衰竭的限制性模式。脑部检查结果显示,78例(80%)被描述为多发性梗死,55例(57%)累及分水岭区域,而11例(11%)被描述为近端一支大血管闭塞所致的栓塞性卒中。关于治疗,分别有71例(73%)、28例(29%)和16例(16%)患者接受了类固醇、抗凝剂和抗血小板治疗。总体死亡率和恢复率分别为11%和89%。医生应该知道,大多数HES相关的卒中病例的特征是分水岭区域的多发性梗死,而且心脏HES并不总是与卒中相关。