Lee Hanbyul, Kim Chang Sin, Jung Jo Won, Park Han Ki, Ahn Jong Gyun
Department of Pediatrics, Soonchunhyang University Seoul Hospital, Soonchunhyang University College of Medicine, Seoul, Korea.
Division of Pediatric Cardiology, Department of Pediatrics, Severance Cardiovascular Hospital, Yonsei University College of Medicine, Seoul, Korea.
Yonsei Med J. 2025 Jun;66(6):390-394. doi: 10.3349/ymj.2023.0186.
Aortic regurgitation (AR) in children is usually caused by congenital valve anomalies, and Behçet syndrome (BS) can be suspected in cases of isolated AR. Patients with BS undergoing aortic valve surgery due to aortic valve invasion have a high risk of complications, such as leakage around the valve and dehiscence. Cardiovascular involvement occurs in 7%-46% of adult patients with BS and is the main cause of mortality; however, its prevalence is unclear and rare in children. A 12-year-old boy was diagnosed with severe AR associated with BS. A progressive subaortic pseudoaneurysm was observed after aortic valve replacement. The periaortic intracardiac pouch was at risk of rupture; therefore, the patient underwent a Bentall operation. After the Bentall procedure, a newly developed subaortic pseudoaneurysm was detected below the prosthetic valve. However, the risk of rupture reduced as the subaortic pseudoaneurysm regressed with anti-inflammatory drugs alone without reoperation. Repeated surgery is inevitable in patients with BS undergoing aortic valve surgery due to the progressive chronic inflammatory reactions that present with a pseudoaneurysm. Here, we report an 8-year follow-up of a pediatric case of BS with subaortic pseudoaneurysm, highlighting the importance of close follow-up, medical management, and early diagnosis in treating this condition.
儿童主动脉瓣反流（AR）通常由先天性瓣膜异常引起，孤立性AR病例可怀疑为白塞病（BS）。因主动脉瓣受累而接受主动脉瓣手术的BS患者发生并发症的风险很高，如瓣膜周围渗漏和裂开。7%-46%的成年BS患者会出现心血管受累，这是主要的死亡原因；然而，其在儿童中的患病率尚不清楚且较为罕见。一名12岁男孩被诊断为与BS相关的严重AR。主动脉瓣置换术后观察到进行性主动脉瓣下假性动脉瘤。主动脉周围心内袋有破裂风险；因此，该患者接受了Bentall手术。在Bentall手术后，在人工瓣膜下方检测到新形成的主动脉瓣下假性动脉瘤。然而，随着主动脉瓣下假性动脉瘤仅通过抗炎药物消退而无需再次手术，破裂风险降低。由于伴有假性动脉瘤的进行性慢性炎症反应，接受主动脉瓣手术的BS患者不可避免地需要重复手术。在此，我们报告一例患有主动脉瓣下假性动脉瘤的儿童BS病例的8年随访情况，强调密切随访、药物治疗和早期诊断在治疗这种疾病中的重要性。
