Department of Cardiac Surgery, Key Laboratory of Pulmonary Vascular Medicine, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 167, Beilishi Road, Beijing, 100037, China.
Department of Cardiovascular Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, No.1277, Jiefang Avenue, Wuhan, 430022, China.
Eur J Med Res. 2023 Aug 7;28(1):269. doi: 10.1186/s40001-023-01239-8.
Isolated pulmonary vasculitis (IPV) is a rare, insidious, and localized inflammatory disease affecting the pulmonary arteries, often leading to severe luminal obstruction. The prognosis for patients with occlusive IPV is poor, and there is currently a lack of effective treatments. The objective of this study was to evaluate the performance of pulmonary endarterectomy (PEA) as a treatment for occlusive IPV.
This single-center retrospective analysis included patients who received PEA for occlusive IPV between January 2018 and June 2022. Clinical characteristics and hemodynamic parameters were evaluated at baseline and follow-up.
Among 114 consecutive patients who underwent PEA, occlusive IPV was identified in 7 patients. Two patients underwent bilateral PEA for the involvement of both pulmonary arteries. Patch angioplasty was performed to treat four severe constrictions. One patient died from residual pulmonary hypertension after limited PEA of a transmural vascular lesion. In addition, no obvious surgical complications were observed. Three months after PEA, a substantial relief in symptoms was achieved. Also, there is a decrease in the mean pulmonary artery pressure (median 33 [20-48] mmHg before versus median 21 [16-26] mmHg after; P < 0.018) and pulmonary arterial resistance (median 234 [131-843] dyn.s.cm versus median 180 [150-372] dyn.s.cm; P = 0.310). Three patients experienced a relapse of restenosis of the treated arteries within a 6-month follow-up period, despite daily oral prednisolone administration. They were treated with balloon pulmonary angioplasty of both the main pulmonary arteries and branches.
PEA is a valuable choice for treating occlusive IPV, with notable hemodynamic and clinical advantages. To increase long-term vascular patency, complete management should be optimized.
孤立性肺动脉炎（IPV）是一种罕见的、隐匿的、局限性炎症性疾病，影响肺动脉，常导致严重的管腔阻塞。闭塞性 IPV 患者的预后较差，目前缺乏有效的治疗方法。本研究旨在评估肺动脉内膜切除术（PEA）治疗闭塞性 IPV 的效果。
这是一项单中心回顾性分析，纳入了 2018 年 1 月至 2022 年 6 月期间因闭塞性 IPV 接受 PEA 治疗的患者。在基线和随访时评估临床特征和血流动力学参数。
在 114 例连续接受 PEA 的患者中，7 例患者被诊断为闭塞性 IPV。2 例患者因累及双侧肺动脉而行双侧 PEA。4 例严重狭窄行补片血管成形术治疗。1 例因局限于透壁血管病变的 PEA 术后残留肺动脉高压死亡。此外，未观察到明显的手术并发症。PEA 后 3 个月，症状明显缓解。平均肺动脉压（PEA 前中位数 33[20-48]mmHg 与 PEA 后中位数 21[16-26]mmHg；P=0.018）和肺动脉阻力（PEA 前中位数 234[131-843]dyn·s·cm 与 PEA 后中位数 180[150-372]dyn·s·cm；P=0.310）均降低。3 例患者在 6 个月的随访期间，尽管每天口服泼尼松龙治疗，仍出现治疗动脉再狭窄复发。他们接受了主肺动脉和分支的球囊肺动脉成形术治疗。
PEA 是治疗闭塞性 IPV 的一种有价值的选择，具有显著的血流动力学和临床优势。为了提高长期血管通畅率，应优化综合管理。
