Grollier G, Montécot L, Galateau F, Scanu P, Gofard M, Hédoire F, Lassaigne D, Maïza M, Maïza D, Potier J C
Service de soins intensifs de cardiologie, CHU, Côte de Nacre.
Arch Mal Coeur Vaiss. 1990 Jun;83(7):1007-10.
A 70 year old woman was admitted for right ventricular failure and cyanosis of recent onset. Echocardiography showed a very large, homogenous, immobile, smooth-contoured mass filling the right atrium. Right atrial pressures were raised but the other intracardiac pressures were normal at catheterisation. Right heart angiography confirmed the voluminous right atrial mass and dilatation of the hepatic veins and showed early opacification of the left heart chambers. A right-to-left shunt was confirmed by oximetry which showed significant desaturation of the blood in the left atrium and ventricle (saturation 78% in the left ventricle). The tumour was also documented by a thoracic CT scan. At surgery, a very large, malignant right atrial tumour was resected which histological examination showed to be an angiosarcoma. The interatrial septum seemed to be intact: there was no true atrial septal defect but a persistent foramen ovale was found. After resection of the tumour the right atrium was reconstructed. The initial postoperative period was uncomplicated but the patient died nine months later of metastatic disease particularly affecting the liver and brain. The clinical presentation of malignant cardiac tumour is very variable but a right-to-left interatrial shunt through a patent foramen ovale has only been reported previously in 2 cases of primary malignant tumours (a rhabdomyosarcoma and an angiosarcoma) and in 1 case of a secondary cardiac metastasis.
一名70岁女性因近期出现右心室衰竭和发绀入院。超声心动图显示右心房内有一个非常大的、均匀的、固定不动的、轮廓光滑的肿块。右心房压力升高,但心导管检查时其他心腔内压力正常。右心造影证实了巨大的右心房肿块以及肝静脉扩张,并显示左心腔早期显影。血氧测定证实存在右向左分流,显示左心房和左心室内血液明显氧饱和度降低(左心室饱和度为78%)。胸部CT扫描也记录了该肿瘤。手术中,切除了一个非常大的恶性右心房肿瘤,组织学检查显示为血管肉瘤。房间隔似乎完整:没有真正的房间隔缺损,但发现有一个持续存在的卵圆孔未闭。肿瘤切除后,对右心房进行了重建。术后初期无并发症,但患者九个月后死于转移性疾病,主要累及肝脏和大脑。恶性心脏肿瘤的临床表现非常多样,但通过卵圆孔未闭的右向左房间分流此前仅在2例原发性恶性肿瘤(1例横纹肌肉瘤和1例血管肉瘤)以及1例继发性心脏转移瘤中报道过。