Prognostic Significance of Computed Tomography Findings in Pulmonary Vein Stenosis

• Main Authors: Laureen Sena, Ryan Callahan, Lynn A. Sleeper, Rebecca S. Beroukhim
• Format: Article
• Language: English
• Published: MDPI AG 2021-05-01
• Series: Children
• Subjects: pulmonary vein stenosis; pulmonary vein atresia; pulmonary vein compression; computed tomography; pulmonary venous collaterals; perihilar induration
• Online Access: https://www.mdpi.com/2227-9067/8/5/402

(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 (<i>p</i> < 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.