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右肺动脉起源于升主动脉异常。

Anomalous origin of the right pulmonary artery from the ascending aorta.

作者信息

Prifti Edvin, Bonacchi Massimo, Murzi Bruno, Crucean Adrian, Leacche Marzia, Bernabei Massimo, Bartolozzi Fabio, Nathan Nadia S, Vanini Vittorio

机构信息

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

出版信息

J Card Surg. 2004 Mar-Apr;19(2):103-12. doi: 10.1111/j.0886-0440.2004.04023.x.

Abstract

OBJECTIVES

The aim was to review our experience with the surgical repair of the anomalous origin of the right pulmonary artery (AORPA) from the aorta.

MATERIALS AND METHOD

Between January 1991 and March 2001, five patients with AORPA underwent surgical correction. One patient presented isolated AOPA from the aorta. Implantation of the anomalous PA to the main PA trunk was performed by (1). direct anatomosis employing an autologous pericardial patch in two patients; (2). using an aortic flap in two patients with AORPA; and (3). using an aortic and pulmonary flaps in another patient. The mean follow-up time was 27 months.

RESULTS

One patient died due to progressive heart failure unresponsive to inotropic support. Early postoperative pulmonary hypertension crisis was identified in patient 4, that was managed by intravenous prostacyclin. The same patient necessitated mechanical ventilation for 11 days. The mean residual gradient for all survivors was 9.5 +/- 4 mmHg. The postoperative Tc-99 m scintigraphy demonstrated 72 +/- 4.5(%) lung perfusion. At follow-up the survival was 100%. None of the followed patients required reoperation.

CONCLUSION

The AORPA from the aorta is a rare but important entity, necessitating a scrupulous preoperative and intraoperative evaluation. The techniques employing autologous tissues for enlarging and lengthening the AORPA seem to be associated with better results in terms of postoperative restenosis.

摘要

目的

回顾我们对主动脉起源的右肺动脉异常(AORPA)进行手术修复的经验。

材料与方法

1991年1月至2001年3月期间,5例AORPA患者接受了手术矫正。1例患者表现为孤立的主动脉起源的AOPA。将异常肺动脉植入主肺动脉干的方法如下:(1)2例患者采用自体心包补片直接吻合;(2)2例AORPA患者采用主动脉瓣片;(3)另1例患者采用主动脉瓣片和肺动脉瓣片。平均随访时间为27个月。

结果

1例患者因对强心支持无反应的进行性心力衰竭死亡。患者4术后早期出现肺动脉高压危象,通过静脉注射前列环素进行处理。该患者需要机械通气11天。所有存活患者的平均残余压差为9.5±4 mmHg。术后Tc-99 m闪烁扫描显示肺灌注为72±4.5(%)。随访时生存率为100%。所有随访患者均无需再次手术。

结论

主动脉起源的AORPA是一种罕见但重要的疾病,术前和术中需要仔细评估。采用自体组织扩大和延长AORPA的技术在术后再狭窄方面似乎效果更好。

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