Bitar Fouad, Bulbul Ziad, Jassar Yehya, Zareef Rana, Abboud Jennifer, Arabi Mariam, Bitar Fadi Fouad
Department of Pediatric and Adolescent, American University of Beirut Medical Center, Beirut 1103, Lebanon.
Department of Surgery, American University of Beirut Medical Center, Beirut 1103, Lebanon.
World J Cardiol. 2024 Oct 26;16(10):595-603. doi: 10.4330/wjc.v16.i10.595.
Unroofed coronary sinus (UCS) is a rare subtype of atrial septal defect. It is frequently associated with a persistent left superior vena cava and is often part of a more intricate cardiac malformation.
This report describes a rare case of an adolescent patient with UCS featuring atrial situs solitus, absence of the right superior vena cava and a persistent left superior vena cava draining into the left atrium consistent with total unroofing of the coronary sinus. This was associated with concurrent severe mitral insufficiency secondary to redundant and prolapsing leaflets, and a substantial left-to-right shunt across the coronary sinus orifice. A comprehensive examination of the existing literature is included, shedding light on the diagnostic challenges of UCS and describing the available surgical options within the context of mitral valve surgery.
UCS is a complex condition requiring careful consideration of associated anomalies and a tailored surgical approach.
冠状静脉窦未闭（UCS）是房间隔缺损的一种罕见亚型。它常与永存左上腔静脉相关，且常是更复杂心脏畸形的一部分。
本报告描述了一例罕见的青少年UCS病例，其心房位置正常，右上腔静脉缺如，永存左上腔静脉引流至左心房，符合冠状静脉窦完全未闭。这与继发于瓣叶冗长和脱垂的重度二尖瓣关闭不全以及经冠状静脉窦口的大量左向右分流同时存在。文中对现有文献进行了全面综述，阐明了UCS的诊断挑战，并描述了二尖瓣手术背景下可用的手术选择。
UCS是一种复杂病症，需要仔细考虑相关异常情况并采取量身定制的手术方法。
