Vida Vladimiro L, Speggiorin Simone, Padalino Massimo A, Crupi Giancarlo, Marcelletti Carlo, Zannini Lucio, Frigiola Alessandro, Varrica Alessandro, Di Carlo Duccio, Di Donato Roberto, Murzi Bruno, Bernabei Massimo, Boccuzzo Giovanna, Stellin Giovanni
Pediatric and Congenital Cardiac Surgery Unit, University of Padua, Padua, Italy.
Ann Thorac Surg. 2009 Aug;88(2):440-4. doi: 10.1016/j.athoracsur.2009.04.099.
Scimitar syndrome is a rare congenital heart disease. To evaluate the results of managing this malformation surgically, we have embarked on a multicenter Italian study involving seven different centers and reporting the largest published series in the medical literature.
From January 1997 to December 2007, 26 patients with scimitar syndrome who underwent surgical correction were included. Primary outcomes include hospital mortality and the efficacy of repair at the follow-up.
Median age was 11 years (interquartile range, 1.8 to 19.9 years). Nineteen patients (73%) presented with symptoms including upper respiratory tract infections (n = 13), recurrent pneumonia (n = 10), cardiac failure (n = 4), and cyanosis (n = 2). Associated cardiac anomalies were present in 16 patients (63%). Surgical repair included intraatrial baffle repair in 18 patients (69%; group 1), and reimplantation of the "scimitar vein" onto the left atrium in 8 patients (31%; group 2). One patient died in hospital (3.8%; group 1). Postoperative complications were less frequently reported in group 1 (4 of 18 patients, 22%) compared with group 2 (5 of 8 patients, 62%). Median follow-up time was 4 years (interquartile range, 1.8 to 9.7 years). There was 1 late death (1/25 patients, 4%; group 2). Four patients (16%) showed a complete occlusion of the scimitar drainage (2 in group 1, 12%; 2 in group 2, 25%) and 3 patients (12%) required balloon dilation or stenting for scimitar vein stenosis (1 in group 1, 6%; 2 in group 2, 25%).
The intraatrial baffle repair seems to have a lower incidence of postoperative complications and a better patency rate, at last follow-up, than the reimplantation of the scimitar vein onto the left atrium.
弯刀综合征是一种罕见的先天性心脏病。为评估手术治疗这种畸形的效果，我们开展了一项意大利多中心研究，涉及七个不同中心，报告了医学文献中已发表的最大系列病例。
纳入1997年1月至2007年12月期间接受手术矫正的26例弯刀综合征患者。主要结局包括住院死亡率和随访时修复的疗效。
中位年龄为11岁（四分位间距为1.8至19.9岁）。19例患者（73%）出现症状，包括上呼吸道感染（n = 13）、反复肺炎（n = 10）、心力衰竭（n = 4）和发绀（n = 2）。16例患者（63%）存在相关心脏异常。手术修复包括18例患者（69%；第1组）进行心房内挡板修复，8例患者（31%；第2组）将“弯刀静脉”重新植入左心房。1例患者在医院死亡（3.8%；第1组）。与第2组（8例患者中的5例，62%）相比，第1组术后并发症报告较少（18例患者中的4例，22%）。中位随访时间为4年（四分位间距为1.8至9.7年）。有1例晚期死亡（25例患者中的1例，4%；第2组）。4例患者（16%）出现弯刀引流完全闭塞（第1组2例，12%；第2组2例，25%），3例患者（12%）因弯刀静脉狭窄需要球囊扩张或支架置入（第1组1例，6%；第2组2例，25%）。
在最后随访时，心房内挡板修复术后并发症发生率似乎低于将弯刀静脉重新植入左心房，且通畅率更高。
