Successful Repair of Infantile Scimitar Syndrome with Contralateral Pulmonary Vein Stenosis in an Infant

Abstract Infantile scimitar syndrome (SS) is a rare congenital heart disease and has high mortality. Guidelines have not been established, but surgery is indicated in symptomatic patients. Despite the various surgical approaches, outcomes continue to be disappointing. We present our surgical experience with an infantile SS patient who had stenotic pulmonary veins contralateral to the hypoplastic lung with complicated anatomy. There are few cases with this complex pathology in the literature. Moreover, our patient was the first transplant-free survivor with this complexity in the literature.

摘要 婴儿弯刀综合征（Infantile scimitar syndrome, SS）是一种罕见的先天性心脏病，病死率较高。目前尚未建立标准化诊疗指南，仅对存在症状的患者推荐手术治疗。尽管已有多种手术方案可供选择，术后预后仍不尽如人意。本文报告1例合并复杂解剖畸形的婴儿弯刀综合征患者，该患者的病变特征为患侧肺发育不良，且发育不良肺的对侧存在肺静脉狭窄。此类伴随复杂病理特征的病例在既往文献中鲜有报道，且本病例为目前文献记载中首例无需移植的该类复杂病症存活患者。