Primary Pulmonary Vein Stenosis: Outcomes, Risk Factors, and Severity Score in a Multicentric Study

Abstract

Primary PVS still has a guarded prognosis in the current era despite adoption of the sutureless technique. Postoperative pulmonary hypertension and severity of disease evaluated by a new severity score are independent prognostic factors regardless of surgical technique.

Outcomes and prognostic factors for postsurgical pulmonary vein stenosis in the current era

“…Of note, female gender was associated with poorer outcomes, which will need to be confirmed by other studies. The intrinsic severity of the disease before or after the procedure appears to be the most decisive factor determining final outcomes, not only in congenital PVS 11,12,16 but also in PSPVS, as shown in the present series. A high postoperative PVS score at the time of hospital discharge is an independent risk factor of PV restenosis, reintervention, and death.…”

“…A greater number of stenotic PVs has been shown to be associated with an increased risk of death, especially when there is bilateral involvement in some series of PSPVS. 19,24,25 The only 5 independent predictors of mortality demonstrated so far in the literature in multivariate analysis (high preoperative PVS score by Viola and colleagues, 12 involvement of greater than 3 PV and progression to uninvolved PVs by Song and colleagues, 11 and postoperative high PVS score and pulmonary hypertension 1 month after surgery by Kalfa and colleagues 16 ) were shown in series focusing on congenital PVS. The multivariable analysis in our series confirms that the severity of the residual disease and the adequacy of the surgical PV decompression at the time of hospital discharge are the main determinants of late outcomes in patients with PSPVS.…”

“…A specific PVS severity score was previously developed by our group. 16 Briefly, the assessment of each vein was based on echocardiography before each procedure in all cases, strengthened by angiography, computed tomography (CT) scan and magnetic resonance imaging (MRI) in 52%, 9%, and 7% of cases, respectively. This score is based on (1) the degree of the stenosis evaluated by the echographic pressure gradient measured at the venoatrial junction, (2) the focal/diffuse aspect of the stenosis evaluated by the combined imaging modalities mentioned above, and (3) the unilateral/bilateral feature of the disease.…”

“…The concept of the sutureless technique for PVS repair avoiding the suture of the veins themselves was introduced in the mid-1990s. [3][4][5][6] This technique was presented as real progress in some early reports, [7][8][9] while appearing disappointing in the more recent ones, [10][11][12][13][14][15][16] especially in the series focusing on patients with congenital PVS. [11][12][13]15,17 The outcomes of this technique in the specific population of PSPVS and risk factors for poor outcomes are still controversial because series are often limited and heterogeneous.…”

Congenital pulmonary vein stenosis at an African tertiary care centre over a 25-year period

“…(13) Histologic series have identified fibroblasts and myofibroblastlike cells as significant contributors to the mesenchymal proliferation that is a hallmark of pulmonary vein stenosis pathophysiology. (2,14) Callahan, et al evaluated the use of imatinib mesylate with or without bevacizumab targeting neoproliferative myofibroblast-like cells with tyrosine kinase receptor expression, as adjuncts to modern interventional therapies for the treatment of multivessel intraluminal pulmonary vein stenosis. (13) The results of the study were inconclusive with a major confounding factor being the use of other conventional management of the PVS during the study period.…”