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左肺动脉起自主动脉的异常情况。我们的经验及文献综述。

Anomalous origin of the left pulmonary artery from the aorta. Our experience and literature review.

作者信息

Prifti Edvin, Bonacchi Massimo, Murzi Bruno, Crucean Adrian, Bernabei Massimo, Luisi Vincenzo Stefano, Bartolozzi Fabio, Vanini Vittorio

机构信息

Department of Pediatric Cardiac Surgery, G. Pasquinucci Hospital, Massa, Italy.

出版信息

Heart Vessels. 2003 May;18(2):79-84. doi: 10.1007/s10380-002-0684-7.

Abstract

Three patients with anomalous origin of the left pulmonary artery (AOLPA) from the aorta underwent surgical correction. Two patients presented with an isolated malformation and one with an associated ventricular septal defect. One of the patients with an isolated malformation presented with an AOLPA from the descending thoracic aorta. Implantation of the AOLPA to the main PA trunk was performed by direct anastomosis in two patients and by interposition of a synthetic graft in the other patient with AOLPA from the descending aorta. All patients survived the operation. Low cardiac output was identified in one patient. The mean residual gradient through the anastomotic site was 21 mmHg at follow-up. We found almost 72 cases with AOLPA reported in the literature with a high mortality rate in patients not undergoing surgery. The most frequently found associated malformation is tetralogy of Fallot and ventricular septal defect. AOLPA from the aorta is a rare but important entity, necessitating a scrupulous preoperative and intraoperative evaluation. Patients with AOLPA can undergo surgical repair with excellent results.

摘要

三名左肺动脉起源于主动脉(AOLPA)的患者接受了手术矫正。两名患者表现为孤立性畸形,一名患者合并室间隔缺损。其中一名孤立性畸形患者的左肺动脉起源于胸降主动脉。两名患者通过直接吻合将左肺动脉植入主肺动脉干,另一名左肺动脉起源于降主动脉的患者则通过植入人工合成移植物进行手术。所有患者均手术存活。一名患者出现低心排血量。随访时通过吻合部位的平均残余压差为21 mmHg。我们在文献中发现了近72例AOLPA病例,未接受手术的患者死亡率很高。最常见的相关畸形是法洛四联症和室间隔缺损。主动脉起源的AOLPA是一种罕见但重要的疾病,需要进行细致的术前和术中评估。AOLPA患者可以接受手术修复,效果良好。

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