Pulmonary endarterectomy in patients with occlusive isolated pulmonary vasculitis

Abstract

Background 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. Methods 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. Results 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−5 versus median 180 [150–372] dyn.s.cm−5; 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. Conclusions 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.

Case report: Pulmonary endarterectomy in Takayasu arteritis patient with group IV pulmonary hypertension: A rare clinical scenario

“…On the contrary, CT imaging revealed partially recanalized pulmonary arteries and there were multiple perfusion defects in her V/Q scan. Although successfully treated cases with primary pulmonary vasculitis were reported by PEA, 6,7 data about TAK patients with Group IV PH is scarce. There are two case reports in the literature; one died shortly after the operation due to heart failure, 8 and the other improved symptomatically and functionally, although this report lacks hemodynamic and V/Q scan evaluation after PEA.…”