Rambusch E G, Chavan A, Harms C P, Tietge U, Ocran K, Strassburg C, Wagner S, Galanski M, Creutzig A, Manns M P
Abteilung Gastroenterologie und Hepatologie, Zentrum Innere Medizin und Dermatologie, Medizinische Hochschule Hannover.
Vasa. 1996;25(4):352-7.
Hereditary hemorrhagic teleangiectasia (HHT) (Osler-Weber-Rendu-disease) is characterized by a combination of muccocutaneous vascular malformations with recurrent spontaneous bleeding and a familiar predisposition. Among visceral manifestations of this disease are only a few descriptions of hepatic involvement. We describe a 53-year-old woman with HHT whose first symptoms of hypercirculatory heart failure developed 1.5 years before the final diagnosis of HHT. We measured a heart-time-volume of 14-151/min. by echocardiography and a flow-volume of 5-9 l/min. in the proper hepatic artery by dopplersonography on admission. We were able to demonstrate multiple intra- and extra-hepatic and gastric arteriovenous malformations by arteriography. Branches of the proper hepatic artery and the left gastric artery were embolized in three serial sessions. By this procedure we were able to reduce the flow-volume in the proper hepatic artery from 5-9l/min. to 1.5l/min. and the heart-time-volume to a minimum of 9.8l/min. and thus stop progression of the hyperdynamic heart failure.
遗传性出血性毛细血管扩张症（HHT）（奥斯勒-韦伯-伦杜病）的特征是皮肤黏膜血管畸形合并反复自发性出血以及家族易感性。在该疾病的内脏表现中，仅有少数关于肝脏受累的描述。我们描述了一名53岁患有HHT的女性，其高循环性心力衰竭的首发症状在HHT最终确诊前1.5年出现。入院时，通过超声心动图测量其心排血量为14 - 15升/分钟，通过多普勒超声检查测量肝固有动脉血流量为5 - 9升/分钟。通过血管造影，我们能够证实肝内外及胃存在多处动静脉畸形。在三个连续疗程中对肝固有动脉分支和胃左动脉进行了栓塞。通过该操作，我们能够将肝固有动脉血流量从5 - 9升/分钟降至1.5升/分钟，将心排血量降至最低9.8升/分钟，从而阻止了高动力性心力衰竭的进展。
