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）是一种罕见的先天性心脏病，病死率较高，目前尚无统一诊疗指南，有症状患者具有手术指征。尽管已有多种手术入路可供选择，但术后预后仍不尽如人意。本文报告1例合并复杂解剖结构异常的婴儿弯刀综合征患者的手术治疗经验，该患者的狭窄肺静脉（stenotic pulmonary veins）与发育不良肺叶呈对侧分布。此类合并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未接受移植便存活的该类复杂病例。