Patient with Behçet Syndrome with Subaortic Pseudoaneurysm Who Underwent the Bentall Operation Showed Improvement after Medical Treatment

Abstract

Aortic regurgitation (AR) in children is usually caused by congenital valve anomalies, and Beh & ccedil;et 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.