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贝里综合征,一种复杂的主肺动脉畸形:新生儿一期修复术

Berry syndrome, a complex aortopulmonary malformation: one-stage repair in a neonate.

作者信息

Abbruzzese P A, Merlo M, Chiappa E, Bianco R, Ferrero F, Cappone C M

机构信息

Dipartimento di Cardiologia e Cardiochirurgia and Servizio di Anestesia e Rianimazione, Ospedale Infantile Regina Margherita, Torino, Italy.

出版信息

Ann Thorac Surg. 1997 Oct;64(4):1167-9. doi: 10.1016/s0003-4975(97)00810-2.

Abstract

Successful one-stage repair of a Berry syndrome (interrupted aortic arch, distal aortopulmonary septal defect, right pulmonary artery branch originating from the ascending aorta, and intact ventricular septum) in the neonatal period has been reported in only 2 cases. We report the case of a newborn operated on with deep hypothermic arrest and isolated myocardial perfusion in whom the interrupted aortic arch was corrected by direct anastomosis between the ascending and descending aorta and the aortopulmonary septal defect was treated with reconstruction of the pulmonary trunk and right pulmonary artery, using a flap of aortic tissue. A native pericardial patch was used to reconstruct the ascending aorta.

摘要

新生儿期成功进行贝里综合征(主动脉弓中断、远端主动脉肺动脉间隔缺损、右肺动脉分支起源于升主动脉且室间隔完整)一期修复的报道仅有2例。我们报告1例新生儿病例,该患儿在深低温停循环和单纯心肌灌注下接受手术,术中通过升主动脉与降主动脉直接吻合矫正主动脉弓中断,并采用主动脉组织瓣重建肺动脉干和右肺动脉,治疗主动脉肺动脉间隔缺损。使用自体心包补片重建升主动脉。

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