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波特分流术与小儿肺动脉高压:我们所了解到的情况。

Potts Shunt and Pediatric Pulmonary Hypertension: What We Have Learned.

作者信息

Grady R Mark, Eghtesady Pirooz

机构信息

Division of Cardiology, Department of Pediatrics, Washington University School of Medicine, St. Louis, Missouri.

Section of Pediatric Cardiothoracic Surgery, Department of Surgery, Washington University School of Medicine, St. Louis, Missouri.

出版信息

Ann Thorac Surg. 2016 Apr;101(4):1539-43. doi: 10.1016/j.athoracsur.2015.08.068. Epub 2015 Oct 28.

Abstract

BACKGROUND

A Potts shunt has been proposed as effective palliative therapy in children with severe pulmonary hypertension (PH) who have suprasystemic right ventricular pressures.

METHODS

A retrospective single-center study was performed to assess outcomes in 5 children who underwent a Potts shunt for severe PH.

RESULTS

All 5 children were in World Health Organization functional class IV. Only 3 children were classified as having idiopathic pulmonary arterial PH. Preoperatively, 4 children were receiving intravenous prostacyclins, and 3 were placed on intravenous inotropes for acute right-side heart failure. Three children were potential lung transplant candidates. All but 1 child had evidence for suprasystemic right heart pressures immediately before their operation. All 5 children survived the procedure without significant complications. Four of the 5 children were successfully discharged from the hospital and have had sustained clinical improvement with follow-up ranging from approximately 5 to 16 months. The child who did not have suprasystemic right-side heart pressures before the operation did not benefit from the Potts shunt.

CONCLUSIONS

The Potts shunt can be an effective palliation for children with severe PH. Our results further suggest that (1) a Potts shunt should be considered early in a child's clinical course, before right ventricular deterioration develops; (2) a Potts shunt should be considered in any child with severe, intractable PH regardless of etiology; (3) one might consider a Potts shunt in lieu of intravenous prostacyclins; and (4) a Potts shunt should be considered before lung transplantation and does not preclude future transplantation candidacy.

摘要

背景

对于患有严重肺动脉高压(PH)且右心室压力超过体循环压力的儿童,Potts分流术已被提议作为一种有效的姑息治疗方法。

方法

进行了一项回顾性单中心研究,以评估5例接受Potts分流术治疗严重PH的儿童的治疗效果。

结果

所有5名儿童均处于世界卫生组织功能分级IV级。只有3名儿童被归类为特发性肺动脉高压。术前,4名儿童接受静脉注射前列环素,3名因急性右侧心力衰竭接受静脉注射正性肌力药物治疗。3名儿童是潜在的肺移植候选者。除1名儿童外,所有儿童在手术前均有右心压力超过体循环压力的证据。所有5名儿童手术成功,无明显并发症。5名儿童中有4名成功出院,随访约5至16个月,临床持续改善。术前右心压力未超过体循环压力的儿童未从Potts分流术中获益。

结论

Potts分流术可为患有严重PH的儿童提供有效的姑息治疗。我们的结果进一步表明:(1)应在儿童临床病程早期、右心室恶化之前考虑Potts分流术;(2)对于任何患有严重、难治性PH的儿童,无论病因如何,均应考虑Potts分流术;(3)可考虑用Potts分流术替代静脉注射前列环素;(4)在肺移植前应考虑Potts分流术,且该手术不排除未来的移植候选资格。

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