Pulmonary vein stenosis (PVS) is a rare but serious condition characterized by obstruction of extrapulmonary segments of pulmonary veins which leads to progressive pulmonary hypertension. Objective. In this study we aimed to determine relationship between clinical features and histopathologic findings in patients with PVS. Material and methods. We retrospectively reviewed 34 consecutive patients who underwent PV stenosis repair. Surgical wedge biopsy specimens were collected intraoperatively in 11 patients and reviewed using light microscopy. Results. Affected pulmonary veins in patients with primary PVS were characterized by diffuse stenosis extending into the lung parenchyma. In post-surgical group stenosis was found in a limited segment of pulmonary vein at its ostium. Microscopically, abnormal intimal proliferation was identified in both patient groups. Scaring was predominant finding in patients with post-surgical PVS. Conclusion. In patients with PVS, pathophysiological mechanism influences the severity and extent of clinical manifestations. A comprehensive understanding of this mechanism may improve results of the treatment.

Pulmonary vein (PV) stenosis is a rare disease with high recurrence and mortality rates. Objective. This study was aimed to evaluate the diagnostic accuracy of echocardiography versus cross-sectional modalities. Material and methods. Our study includes 11 consecutive patients who underwent a comprehensive PV anatomy assessment between April 2018 and June 2019 at Ukrainian Children’s Cardiac Center, Kyiv. Echocardiographic studies of each pulmonary vein followed by computed tomography (CT) or magnetic resonance imaging (MRI) were performed in all patients. A total of 27 CT/MRI studies were performed. Results. There were four patients whose echocardiography findings were completely confirmed by CT/MRI method. In some patients significant stenosis was underestimated by echocardiography. Sensitivity and specificity were 86.4 and 88.9 for echo, as well as 100 and 94.4 for CT/MRI, respectively. Conclusion. This study recognizes the limitations of echocardiography as an imaging tool for pulmonary veins morphology assessment. Cross-sectional studies overcome these limitations and provide excellent morphological as well as functional (MRI) evaluation of pulmonary veins.