孤立性肺动脉高安动脉炎术后管理中的陷阱：1例肺动脉移植置换术后34个月的经验报告

Pitfalls in the management of isolated pulmonary Takayasu's arteritis after surgery: a case report of an experience during 34 months after a pulmonary artery graft replacement.

作者信息

Fujita Kishu, Kasama Shu, Kurabayashi Masahiko

机构信息

Department of Cardiovascular Surgery, Hokkaido Ohno Hospital, 1-1-30 Nishino 4 Jyou, Chuo-ku, Sapporo, Hokkaido,, 063-0034, Japan.

Department of Medicine and Biological Science (Cardiovascular Medicine), Gunma University Graduate School of Medicine, 3-39-22 Showa-machi, Maebashi, Gunma, 371-8511, Japan.

出版信息

J Cardiothorac Surg. 2016 Jan 16;11:7. doi: 10.1186/s13019-016-0413-3.

DOI:10.1186/s13019-016-0413-3

PMID:26772757

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4715312/

Abstract

BACKGROUND

Several controversial matters still remain unresolved in the management of Takayasu's arteritis, especially after vascular intervention. First, a definitive diagnostic tool has not been established to assess disease activity correctly. Second, the optimal medical regimen has not been established to prevent restenosis of the vascular lesion. Surgical treatments have been rarely performed to relieve critical vascular stenosis in isolated pulmonary Takayasu's arteritis, but their postoperative courses on long-term follow-up periods have not been sufficiently reported.

CASE PRESENTATION

A 48-year-old man underwent a successful graft replacement for severe right main pulmonary artery stenosis due to isolated pulmonary Takayasu's arteritis. The patient had remained asymptomatic with no clinical inflammatory signs under adequate anticoagulation therapy since then. However, stenosis of the prosthetic graft accompanied by marked pulmonary hypertension was detected 18 months after surgery. Anti-inflammatory treatment with only 5 mg/day of oral prednisolone was then implemented, and the stenosis remained unchanged with the patient being stable for the next 16 months.

CONCLUSIONS

This is the first published case report that describes the actual clinical course with a long-term follow-up period after surgery for isolated pulmonary Takayasu's arteritis, including images of the stenosed prosthetic graft. This case suggests that patients should be followed with multiple complementary diagnostic techniques on the assumption that restenosis is highly possible and unpredictable even after surgery. Besides, sufficient anti-inflammatory treatment should be applied as soon as possible after surgery no matter how inactive the disease appears to be, although its optimal regimen especially during the inactive inflammatory phase needs to be further established.

摘要

背景

在大动脉炎的治疗中，尤其是血管介入治疗后，仍有几个有争议的问题尚未解决。首先，尚未建立一种明确的诊断工具来正确评估疾病活动度。其次，尚未确定预防血管病变再狭窄的最佳药物治疗方案。对于孤立性肺动脉大动脉炎导致的严重血管狭窄，很少进行手术治疗以缓解症状，但关于其术后长期随访过程的报道并不充分。

病例介绍

一名48岁男性因孤立性肺动脉大动脉炎导致严重的右主肺动脉狭窄，接受了成功的移植血管置换术。自那时起，在充分的抗凝治疗下，患者一直无症状，也没有临床炎症体征。然而，术后18个月检测到人工血管狭窄并伴有明显的肺动脉高压。随后实施了仅口服泼尼松龙5毫克/天的抗炎治疗，在接下来的16个月里，狭窄情况未变，患者病情稳定。

结论

这是首例发表的病例报告，描述了孤立性肺动脉大动脉炎手术后长期随访的实际临床过程，包括狭窄人工血管的影像。该病例表明，即使在手术后，也应假设再狭窄很可能发生且不可预测，对患者采用多种辅助诊断技术进行随访。此外，无论疾病看起来多么不活跃，术后都应尽快进行充分的抗炎治疗，尽管其最佳治疗方案，尤其是在炎症非活动期，仍需进一步确定。