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室间隔缺损合并严重肺血管阻力升高情况下肺动脉环缩术的评估

Evaluation of pulmonary artery banding in the setting of ventricular septal defects and severely elevated pulmonary vascular resistance.

作者信息

Khan Sadaf A, Gelb Bruce D, Nguyen Khanh H

机构信息

Division of Pediatric Cardiology, Mount Sinai School of Medicine, New York, NY 10029, USA.

出版信息

Congenit Heart Dis. 2006 Sep;1(5):244-50. doi: 10.1111/j.1747-0803.2006.00043.x.

Abstract

BACKGROUND

Children with pulmonary hypertension secondary to large left-to-right, post-tricuspid valve shunts can eventually have severe and/or irreversible pulmonary vascular disease, yielding them inoperable for conventional surgery. It has been shown, however, that unloading of the pulmonary hypertension can result in remodeling of the pulmonary vasculature and, thus, improvement of the pulmonary hypertension.

METHODS

This study explored whether such patients might experience a significant reduction in pulmonary vascular resistance (PVR) after pulmonary artery band (PAB) placement. Pulmonary hypertension hemodynamics were evaluated by cardiac catheterization in 4 patients with pulmonary hypertension secondary to nonrestrictive left-to-right, post-tricuspid valve shunts before and after PAB placement. Two patients with severe pulmonary hypertension who were considered high risk for conventional surgery benefited from PAB placement with a significant reduction in their PVR, permitting subsequent complete intracardiac repair.

RESULTS

The medium-term follow-up for these 2 patients demonstrated good outcomes. The PVR failed to improve after PAB placement in the remaining 2 patients, leading to medical therapy for pulmonary hypertension. There was 1 late death, presumably related to pulmonary hypertension. Current practice provides 3 relatively unattractive options for patients with severe pulmonary hypertension secondary to nonrestrictive left-to-right, post-tricuspid valve shunts: transplantation, high-risk intracardiac repair, or palliative medical therapy.

CONCLUSION

Our study suggests that a staged approach with initial PAB placement can be considered for select patients with large left-to-right, post-tricuspid valve shunts and high PVR prior to committing them to other high-risk therapeutic options.

摘要

背景

继发于大型左向右分流(三尖瓣后分流)的肺动脉高压患儿最终可能会出现严重和/或不可逆的肺血管疾病,导致他们无法进行传统手术。然而,研究表明,减轻肺动脉高压可导致肺血管重塑,从而改善肺动脉高压。

方法

本研究探讨了此类患者在放置肺动脉束带(PAB)后肺血管阻力(PVR)是否会显著降低。通过心脏导管检查评估了4例继发于非限制性左向右、三尖瓣后分流的肺动脉高压患者在放置PAB前后的肺动脉高压血流动力学。2例被认为传统手术风险高的重度肺动脉高压患者受益于PAB放置,PVR显著降低,随后得以进行完全的心内修复。

结果

这2例患者的中期随访显示预后良好。其余2例患者在放置PAB后PVR未改善,因此接受了肺动脉高压的药物治疗。有1例晚期死亡,可能与肺动脉高压有关。目前的治疗方法为继发于非限制性左向右、三尖瓣后分流的重度肺动脉高压患者提供了3种相对不理想的选择:移植、高风险的心内修复或姑息性药物治疗。

结论

我们的研究表明,对于部分大型左向右、三尖瓣后分流且PVR高的患者,在选择其他高风险治疗方案之前,可考虑先进行PAB放置的分期治疗方法。

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