Paediatric Cardiology Division, Department of Pediatrics, King Abdulaziz University Hospital, Jeddah, Saudi Arabia.
Cardiac Surgery Division, Department of Surgery, King Abdulaziz University, Jeddah, Saudi Arabia.
Echocardiography. 2021 Dec;38(12):2095-2099. doi: 10.1111/echo.15263. Epub 2021 Nov 29.
Aortic aneurysms are rare in the pediatric age group and are commonly caused by genetic disorders associated with vasculopathy, weakness and fragility of arterial walls with progressive dilatation or even rupture. We reported a giant aortic aneurysm involving the ascending aorta and aortic arch in a 20-month-old girl with autosomal recessive cutis laxa type 1B (ARCL1B) who presented with hemorrhagic pericardial effusion and tamponade (impending rupture). Successful surgical repair has been done through excision of the aneurysmal part and replacement by Hemashield graft with preservation of the aortic valve.
儿童期的主动脉瘤较为罕见，通常由与血管病变、动脉壁脆弱性和渐进性扩张甚至破裂相关的遗传疾病引起。我们报告了一例 20 个月大的女孩患有常染色体隐性皮肤松弛症 1B 型（ARCL1B），其表现为出血性心包积液和填塞（即将破裂），伴有升主动脉和主动脉弓的巨大主动脉瘤。通过切除动脉瘤部分并用 Hemashield 移植物置换，同时保留主动脉瓣，成功进行了手术修复。
