Grollier G, Galateau F, Scanu P, Commeau P, Voglimacci M, Bernard J P, Khayat A, Potier J C
Service de soins intensifs de cardiologie, CHU Côte de Nacre, Caen.
Arch Mal Coeur Vaiss. 1990 Apr;83(4):561-4.
The authors report the case of a 63 year old woman admitted to hospital for recurrent refractory ventricular tachycardia. Echocardiography and cardiac scintigraphy showed global left ventricular function. Ventriculography confirmed the left ventricular dysfunction and also showed a localised aneurysm of the anterior left ventricular wall. Surgical resection of the aneurysm and an encircling endocardial ventriculotomy were performed but the patient died of a low output syndrome. Pathological examination of the excised tissue showed granulomatous lesions associated with fibrosis interrupting the striated myocardial bundles. The granulomata consisted in a large number of epithelioid histiocytes and very large giant cells with many nuclei. The diagnosis made retrospectively was that of cardiac sarcoidosis causing a ventricular aneurysm and global left ventricular dysfunction. The diagnosis of cardiac sarcoidosis is difficult in the absence of systemic extracardiac involvement because the clinical manifestations and complementary investigations are non specific. The diagnosis may be made by endomyocardial biopsy in 25 per cent of cases, thereby leading to specific treatment with steroids which is sometimes effective.
作者报告了一名63岁女性因反复难治性室性心动过速入院的病例。超声心动图和心脏闪烁扫描显示左心室整体功能。心室造影证实了左心室功能障碍，并显示左心室前壁有局部动脉瘤。对动脉瘤进行了手术切除并实施了环行心内膜心室切开术，但患者死于低输出量综合征。对切除组织的病理检查显示肉芽肿性病变伴有纤维化，中断了横纹肌心肌束。肉芽肿由大量上皮样组织细胞和含有许多细胞核的非常大的巨细胞组成。回顾性诊断为心脏结节病导致心室动脉瘤和左心室整体功能障碍。在没有系统性心脏外受累的情况下，心脏结节病的诊断很困难，因为临床表现和辅助检查都不具有特异性。在25%的病例中可通过心内膜活检做出诊断，从而得以进行有时有效的类固醇特异性治疗。
