Mourad Faisal, Tang Augustine
Lancashire Cardiac Center, Blackpool Victoria Hospital, Blackpool, Lancashire, UK.
J Cardiothorac Surg. 2010 Mar 26;5:16. doi: 10.1186/1749-8090-5-16.
Blau syndrome is a rare granulomatous disorder inherited in an autosomal dominant manner characterized by the early appearance of granulomatous arthritis, skin rash and anterior uveitis. There are very few data on the cardiovascular manifestations of Blau syndrome. Here we report the first case of sinus of valsava aneurysm in Blau syndrome. In isolated unruptured aneurysms of a sinus of Valsalva without compromise of the aortic valve and/or the coronary ostia, repair may be accomplished by simple placation of the aneurysm or excision of the aneurysm(s) and patch closure of the defect(s) between the aortic annulus and the sinu-vascular ridge. Because of the particular conditions in our case, the repair was performed with replacement of the aortic valve and root using a composite graft employing a modified Bentall's technique.
布劳综合征是一种罕见的以常染色体显性方式遗传的肉芽肿性疾病，其特征为早期出现肉芽肿性关节炎、皮疹和前葡萄膜炎。关于布劳综合征心血管表现的数据非常少。在此，我们报告布劳综合征首例瓦氏窦瘤病例。对于孤立的未破裂瓦氏窦瘤，若未累及主动脉瓣和/或冠状动脉开口，可通过简单的动脉瘤折叠术或切除动脉瘤并修补主动脉瓣环与窦血管嵴之间的缺损来完成修复。由于我们病例的特殊情况，采用改良的Bentall技术使用复合移植物进行主动脉瓣和根部置换来进行修复。
