Yuan Dou
Department of Cardiovascular Surgery, Chengdu Shang Jin Nan Fu Hospital, West China Hospital of Sichuan University, China.
J Int Med Res. 2025 Apr;53(4):3000605251332907. doi: 10.1177/03000605251332907. Epub 2025 Apr 30.
Behçet's disease, also known as the oral-ocular-genital syndrome, is a chronic systemic vasculitis characterized by recurrent oral and genital ulcers as well as ocular manifestations such as uveitis and conjunctivitis. When the cardiovascular system is predominantly affected, the condition is known as cardiac Behçet's disease. This variant can manifest as aortic valve regurgitation, conduction system disorders, myocarditis, and even acute myocardial infarction. Aortic regurgitation is an exceedingly rare and threatening cardiovascular complication in patients with Behçet's disease, often indicating a poor prognosis. Surgical intervention for aortic regurgitation in patients with Behçet's disease is associated with high risks of postoperative complications and mortality. To mitigate these risks, techniques such as the Bentall procedure have been employed. This report describes the case of a patient with Behçet's disease and severe aortic regurgitation who underwent the Bentall procedure. Unfortunately, the patient developed a pseudoaneurysm and prosthetic valve detachment at the aortic root 3 months postoperatively. This severe complication necessitated a subsequent intervention utilizing the Cabrol technique for correction.
白塞病,又称口-眼-生殖器综合征,是一种慢性全身性血管炎,其特征为复发性口腔溃疡和生殖器溃疡以及葡萄膜炎和结膜炎等眼部表现。当心血管系统主要受累时,这种情况被称为心脏白塞病。这种变体可表现为主动脉瓣反流、传导系统紊乱、心肌炎,甚至急性心肌梗死。主动脉反流是白塞病患者极为罕见且具有威胁性的心血管并发症,常提示预后不良。白塞病患者主动脉反流的手术干预与术后并发症和死亡率的高风险相关。为降低这些风险,已采用如Bentall手术等技术。本报告描述了一名患有白塞病和严重主动脉反流的患者接受Bentall手术的病例。不幸的是,患者在术后3个月出现了主动脉根部假性动脉瘤和人工瓣膜脱离。这种严重并发症需要随后采用Cabrol技术进行干预以纠正。