摘要
4例女性Berry综合征患儿,平均手术年龄为28.8 d,平均手术体重为3.64 kg,仅病例4是34 W+5早产儿,遂保守治疗至71 d行手术治疗,余3例均在新生儿期完成一期矫治手术,术后全部存活。采用两种不同的外科矫正技术用于修复主-肺动脉窗和异常起源的右肺动脉,包括1例行主动脉内挡板技术,3例行右肺动脉离断再植术(其中1例原位重建,2例右肺动脉前移到主动脉前方重建)。病例2使用主动脉内挡板术的患儿因术后右肺动脉狭窄经历了两次再次手术。病例3和病例4使用右肺动脉前移重建后的患儿随访右肺动脉发育均良好。Berry综合征通过心脏彩色超声和CT血管造影确诊后,应尽快在有经验的儿童心脏中心进行一期手术根治治疗,手术虽然高风险且复杂,但安全有效。
All four patients were female,with an average age of 28.8 days and an average weight of 3.64 kg.Only case 4 was born prematurely at 34 W+5 and was treated conservatively until 71 days to complete operation.All the others completed primary corrective surgery in the neonatal period,and all survived after operation.Two different surgical techniques were used to repair the aortic-pulmonary window and the aortic origin of the right pulmonary artery,including 1 case using the aortic internal baffle technique and another 3 cases underwent replantation of the right pulmonary artery(1 case was reconstructed in situ,and the other 2 cases were reconstructed by moving the right pulmonary artery in the anterior of aorta).Case 2 who received aortic internal baffle technique underwent two reoperation because of right pulmonary artery stenosis.While,right pulmonary artery of cases 3 and 4 developed well after being reconstructed the right pulmonary artery anterior translocation.After the diagnosis of Berry syndrome through cardiac color ultrasound and CT angiography,a primary surgical radical treatment should be performed as soon as possible at an experienced pediatric cardiac center.Although the surgery is high-risk and complex,it is safe and effective.
作者
施旭聪
翁建彬
俞劲
马晓辉
石卓
俞建根
范祥明
SHI Xucong;WENG Jianbin;YU Jin;MA Xiaohui;SHI Zhuo;YU Jiangen;FAN Xiangming(Department of Cardiac Surgery,Children's Hospital,Zhejiang University School of Medicine,National Clinical Research Center for Child Health,Hangzhou,310052,P.R.China;Department of Neurosurgery,Children's Hospital,Zhejiang University School of Medicine,National Clinical Research Center for Child Health,Hangzhou,310052,P.R.China;Department of Ultrasound Diagnosis,Children's Hospital,Zhejiang University School of Medicine,National Clinical Research Center for Child Health,Hangzhou,310052,P.R.China;Department of Radiology,Children's Hospital,Zhejiang University School of Medicine,National Clinical Research Center for Child Health,Hangzhou,310052,P.R.China)
出处
《中国胸心血管外科临床杂志》
北大核心
2025年第11期1672-1676,共5页
Chinese Journal of Clinical Thoracic and Cardiovascular Surgery
基金
浙江省自然科学基金会项目(LY23H020007)。
关键词
Berry综合征
一期矫治
右肺动脉前移重建
肺动脉狭窄
病例报告
Berry syndrome
primary correction
right pulmonary artery anterior translocation
pulmonary artery stenosis
case report
