Department of Cardiothoracic Surgery, Rady Children's Hospital-San Diego, San Diego, CA, USA.
UC San Diego School of Medicine, San Diego, CA, USA.
World J Pediatr Congenit Heart Surg. 2020 Jul;11(4):NP182-NP185. doi: 10.1177/2150135118769418. Epub 2018 Oct 8.
Ehlers-Danlos syndrome vascular type IV is characterized by translucent skin, easy bruising, and fragility of arteries. A full-term female infant presented at four weeks of age with a diagnosis of d-transposition of the great arteries with restrictive atrial septal defect. She successfully underwent emergent balloon atrial septostomy and placement of patent ductus arteriosus (PDA) stent. She required restenting of the PDA and pulmonary artery banding prior to arterial switch procedure. At 16 months of age, the patient successfully underwent arterial switch procedure without complication. This report demonstrates the feasibility of an arterial switch operation along with long-term follow-up of this rare condition.
Ehlers-Danlos 综合征血管型 IV 型的特征为皮肤半透明、容易瘀伤和动脉脆弱。一名足月女性婴儿在四周大时被诊断为大动脉转位伴限制性房间隔缺损。她成功接受了紧急球囊房间隔造口术和动脉导管未闭（PDA）支架置入术。她在进行大动脉调转手术前需要再次放置 PDA 支架和肺动脉带。在 16 个月大时，该患者成功进行了大动脉调转手术，无并发症。本报告展示了这种罕见情况的动脉调转手术的可行性以及长期随访结果。
