Cardiac Primary Prevention Research Center, Cardiovascular Diseases Research Institute, Tehran University of Medical Sciences, Tehran, Iran.
Cardiovascular Surgery Department, Razavi Hospital, Mashhad, Iran.
BMC Cardiovasc Disord. 2024 Nov 1;24(1):610. doi: 10.1186/s12872-024-04298-9.
Marfan syndrome (MFS) is a connective tissue disorder that can lead to cardiovascular and musculoskeletal abnormalities. Aortic aneurysms and dissections are frequently seen in patients with MFS whereas peripheral vascular aneurysms in subclavian and axillary arteries territory considered very unusual. We reported a case of 54-year-old female with known history of MFS who had undergone a mechanical valve Bentall procedure due to severe aortic regurgitation and ascending aorta aneurysm in addition to thoracoabdominal aortic repair and who presented with a pulsatile painful mass in her right axillary region that turn to be significant true aneurysms of right subclavian and axillary arteries. To relive symptoms and to avoid further complications patient underwent successful surgical repair. Our case demonstrated rare locations of true peripheral aneurysms as a possible manifestation of MFS appeared several years post Bentall procedure and thoracoabdominal aortic repair and highlights also the importance of long-term monitoring to detect earlier such manifestation and avoid complications by surgical repair.
马凡综合征（MFS）是一种结缔组织疾病，可导致心血管和肌肉骨骼异常。马凡综合征患者常出现主动脉瘤和夹层，而锁骨下动脉和腋动脉区域的外周血管动脉瘤则非常罕见。我们报告了一例 54 岁女性病例，该患者已知患有 MFS 病史，因严重主动脉瓣反流和升主动脉瘤而行机械瓣 Bentall 手术，此外还进行了胸腹主动脉修复，患者右侧腋窝出现搏动性疼痛性肿块，证实为右锁骨下动脉和腋动脉真性动脉瘤。为缓解症状并避免进一步并发症，患者成功接受了手术修复。我们的病例显示，真性外周动脉瘤的罕见部位可能是 Bentall 手术后和胸腹主动脉修复后数年出现的 MFS 表现之一，这也强调了长期监测的重要性，以便及早发现此类表现并通过手术修复避免并发症。
