PDF(Pubmed)
Abstract：
Granulomatosis with polyangiitis (GPA) is a multi-system disorder associated with ocular, renal, cardiac, pulmonary involvement. However cardiac involvement is very rare compared to other systems. We present a case of an unusual presentation of GPA associated with ocular involvement super imposed on pre-existing rheumatic valvular disease. The cardiac involvement manifested as a thick fibro elastic membrane encasing the aortic and mitral valve annuli making the valve replacements a real technical challenge. The patient needed permanent pacemaker implantation due to intermittent complete heart block that was present even preoperatively. The patient was started on steroids and is doing well until the last follow-up. This case highlights the importance of recognition of GPA in cardiac surgery and to be aware of such an entity when one encounters a thick whitish avascular fibro elastic membrane encasing cardiac valves and endocardium, obliterating the anatomical delineation of structures during surgery.
摘要：
肉芽肿性多血管炎（GPA）是一种与眼部相关的多系统疾病，肾,心脏,肺部受累。然而，与其他系统相比，心脏受累非常罕见。我们介绍了一例与先前存在的风湿性瓣膜病叠加的眼部受累相关的GPA异常表现。心脏受累表现为包裹主动脉和二尖瓣环的厚纤维弹性膜，这使得瓣膜置换成为一项真正的技术挑战。患者需要永久性起搏器植入，因为即使在术前也存在间歇性完全性心脏传导阻滞。患者开始使用类固醇，并且在最后一次随访之前表现良好。这个案例强调了在心脏外科手术中认识GPA的重要性，并在遇到包裹心脏瓣膜和心内膜的厚白色血管纤维弹性膜时意识到这种实体。在手术过程中消除结构的解剖轮廓。
