摘要
目的探讨Berry综合征不同手术方式的差异性以及术后并发症的发生率。方法回顾性分析2003年1月至2017年12月上海交通大学医学院附属上海儿童医学中心收治的19例Berry综合征患儿临床资料,所有患儿均接受一期手术治疗。男12例,女7例;6例新生儿。主动脉弓中断A型17例,B型2例。主肺动脉窗分型中,Ⅱa型6例,Ⅱb型11例,Ⅲ型2例。4例术前因重症肺炎、心功能衰竭接受呼吸机辅助通气。所有患儿均行正中开胸手术,对于主肺动脉窗及右肺动脉起源于升主动脉的纠治,8例通过直接内隧道补片分隔;6例将右肺动脉从升主动脉处剪下,重新移植至肺动脉总干上,并用补片关闭主肺动脉间隔缺损;5例将右肺动脉开口沿着主动脉后壁一起剪下至主肺动脉间隔缺损处,升主动脉行端端吻合。所有患儿均将离断的降主动脉重新与主动脉弓行端侧吻合,前壁用心包补片扩大。结果全组体外循环79~260 min,平均(146.7±63.5)min;主动脉阻断46~179 min,平均(74.3±27.4)min。术后死亡3例,1例术后26天死于术后重症肺炎、多脏器功能衰竭,1例术后1天死于严重低心排血量综合征,1例术后2个月死于心功能不全。术后3例出现肺动脉高压危象。再手术3例,原为右肺动脉开口狭窄2例,再次行补片扩大术;升主动脉压迫右肺动脉1例,再次行Lecompte转位术。结论Berry综合征的术后近、远期病死率偏高,一旦发现应尽早行手术治疗。主肺动脉窗Ⅱa型患者适合用内隧道补片方法重新分隔右肺动脉至肺动脉总干侧,但其疗效仍需中远期随访观察。术中及术后需密切观察是否出现肺动脉高压危象。右肺动脉残余梗阻是再手术的主要原因。
Objective The purpose of this manuscript was to compare the outcomes of different surgical methods of Berry syndrome and to figure out the occurrence of postoperative morbidity.Methods From January 2003 through December 2017, nineteen infants with Berry syndrome underwent one-stage repair at Shanghai Children’s Medical Center. There were 12 male and 7 female. Among them, six patients were neonates. The IAA morphology was type A in 17 patients and type B in 2 patients. The APW morphology was type Ⅱa in 6 patients, type Ⅱb in 11 patients, and type Ⅲ in 2 patients. Preoperative mechanical ventilation was required in 4 patients. Three different surgical correction techniques were employed to repair the APW and AORPA, including intra-aortic baffle in 8 patients, right pulmonary artery(RPA) detachment in 6 patients, and RPA angioplasty with aortic cuff in 5 patients. The descending aorta was then anastomosed to the aortic arch by an end-to-side anastomosis with a patch augmentation in the anterior wall.Results The mean CPB and aortic cross-clamp time was(146.7±63.5)minutes(range, 79 to 260 minutes) and(74.3±27.4)minutes(range, 46 to 147 minutes), respectively. There were 3 deaths. One patient died of severe pneumonia and multi-organ dysfunction on postoperative day 26. One patient suffered severe low cardiac output syndrome after surgery and died on postoperative day 1. One patient died of congestive heart failure at 2 months after discharge. Reoperations were required in 3 patients during the follow-up period. RPA arterioplasty with bovine pericardial patch augmentation was performed in 2 patients for RPA restenosis. Lecompte maneuverer was performed in 1 patient to release the compression of the RPA from the ascending aorta.ConclusionThe mortality of one-stage repair of Berry syndrome was high. Surgical correction should be performed as soon as diagnosed. An intra-aortic baffle patch is suitable for type Ⅱa APW defect patients beyond the neonatal period. Pulmonary hypertension crisis is important after su
作者
胡仁杰
张文
刘鑫荣
董卫
张海波
朱宏斌
Hu Renjie;Zhang Wen;Liu Xinrong;Dong Wei;Zhang Haibo;Zhu Hongbin(Department of Cardiovascular and Thoracic Surgery,Shanghai Children's Medical Center,Shanghai Jiaotong University School of Medicine,Shanghai 200127,China)
出处
《中华胸心血管外科杂志》
CSCD
北大核心
2018年第12期713-716,共4页
Chinese Journal of Thoracic and Cardiovascular Surgery
关键词
心脏缺损
先天性
心脏外科手术
主肺动脉窗
右肺动脉起源于升主动脉
主动脉弓中断
Heart defects, congenital
Cardiac surgical procedunes
Aortopulmonary window
Aortic origin of the right pulmonary artery
Interrupted aortic arch
