Nakamura Yuki, Shikata Fumiaki, Ryugo Masahiro, Okamura Toru, Yasugi Takumi, Izutani Hironori
Department of Cardiovascular Surgery, Ehime University Hospital, Shitsukawa, Toon, Ehime, 791-0295, Japan,
Surg Today. 2014 Dec;44(12):2378-81. doi: 10.1007/s00595-014-0876-6. Epub 2014 Mar 20.
Hereditary hemorrhagic telangiectasia (HHT) is caused by an autosomal dominant gene and characterized by multiple arteriovenous malformations in several organs, leading to bleeding or shunting. These patients often suffer severe infections and heart failure, which should be managed in the perioperative period, when open heart surgery is indicated. We report a case of successful aortic root replacement for active prosthetic valve endocarditis and ventricular septal perforation in a patient with HHT, who had severe heart failure.
遗传性出血性毛细血管扩张症(HHT)由常染色体显性基因引起,其特征是多个器官出现多发性动静脉畸形,导致出血或分流。这些患者常遭受严重感染和心力衰竭,在需要进行心脏直视手术的围手术期应加以处理。我们报告一例患有严重心力衰竭的HHT患者成功进行主动脉根部置换术治疗活动性人工瓣膜心内膜炎和室间隔穿孔的病例。