Prognostic Significance of Computed Tomography Findings in Pulmonary Vein Stenosis

Abstract

(1) Pulmonary vein stenosis (PVS) can be a severe, progressive disease with lung involvement. We aimed to characterize findings by computed tomography (CT) and identify factors associated with death; (2) Veins and lung segments were classified into five locations: right upper, middle, and lower; and left upper and lower. Severity of vein stenosis (0–4 = no disease–atresia) and lung segments (0–3 = unaffected–severe) were scored. A PVS severity score (sum of all veins + 2 if bilateral disease; maximum = 22) and a total lung severity score (sum of all lung segments; maximum = 15) were reported; (3) Of 43 CT examinations (median age 21 months), 63% had bilateral disease. There was 30% mortality by 4 years after CT. Individual-vein PVS severity was associated with its corresponding lung segment severity (p < 0.001). By univariate analysis, PVS severity score >11, lung cysts, and total lung severity score >6 had higher hazard of death; and perihilar induration had lower hazard of death; (4) Multiple CT-derived variables of PVS severity and lung disease have prognostic significance. PVS severity correlates with lung disease severity.

Incidence and Predictors of In-stent Restenosis Following Intervention for Pulmonary Vein Stenosis due to Fibrosing Mediastinitis

“…[23] Additionally, it was shown that PVS severity correlated with the severity of the corresponding lung segment in PVS in children. [24]…”

“…[23] Additionally, it was shown that PVS severity correlated with the severity of the corresponding lung segment in PVS in children. [24] Either over-or underinflation of the balloon or stent may affect restenosis, which has been demonstrated in coronary artery intervention. [25][26][27] Earlier studies documented some factors of restenosis after percutaneous coronary intervention, including vessel size, maximal balloon pressure, stent type, final diameter stenosis, DM, etc.…”

Pulmonary Vein Stenosis: A Review

“…Common manifestations of PVS due to multiple diseases on CT are parenchymal changes (eg, consolidation, "ground glass" opacities, interlobular septal thickening, and volume loss). [33,79,93] Pulmonary vascular changes (eg, oligemia, slowed flow, and collaterals) are also present in patients with PVS. [93] Typical FM dyad/triad/tetralogy is also observed in patients with FM-PVS.…”

“…Its severity depends on the number of affected vessels, stenosis site and degree, and comorbidities. [2,79] Single or mild PVS is always asymptomatic during its early stage. In contrast, exertional dyspnea, hemoptysis, PH, and RHF are common in the late PVS stage or severe cases.…”

Management of Pediatric Pulmonary Vein Stenosis

“…33 Further, CTA allows for the application of a scoring system of PVS severity that has been demonstrated to correlate well with lung disease severity and with PVS-related mortality. 34,35 Cardiac magnetic resonance imaging Cardiac magnetic resonance (CMR) imaging is less commonly employed as a screening or surveillance tool in the management of PVS. It is limited by the duration of the examination, the need for sedation, artifact from previous stent implantation as well as the limited spatial resolution it provides, particularly in smaller patients.…”