Unilateral pulmonary vein atresia: Difficulties of radiological diagnosis

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Abstract

Pulmonary vein atresia is a rare congenital abnormality that could manifest in isolation or in association with other congenital abnormalities in the cardiovascular system such as pulmonary vein hypoplasia. Pulmonary vein atresia leads to changes in cardiovascular functioning. This abnormality is often diagnosed in children with recurrent pneumonia and hemoptysis. In adulthood, pulmonary vein atresia is much less common, with clinical symptoms such as dyspnea during physical exercises and hemoptysis. However, some patients are asymptomatic. Owing to the nonspecific imaging findings, lung parenchymal changes are often misdiagnosed as another lung disease, including inflammatory genesis disease. In this article, a case of a young man with asymptomatic unilateral pulmonary vein atresia combined with pulmonary artery hypoplasia and interstitial lung changes in a lung with hypoplasia was presented. These pathologies were first identified in a 21-year-old patient by contrast-enhanced computed tomography.

About the authors

Veronika V. Zharikova

Moscow City Oncological Hospital No. 1

Author for correspondence.
Email: ZharikovaVV@zdrav.mos.ru
ORCID iD: 0009-0007-1659-8325
Russian Federation, Moscow

Valentin A. Nechaev

Moscow City Oncological Hospital No. 1

Email: NechaevVA1@zdrav.mos.ru
ORCID iD: 0000-0002-6716-5593
SPIN-code: 2527-0130

MD, Cand. Sci. (Medicine)

Russian Federation, Moscow

Evgenia A. Kulikova

Moscow City Oncological Hospital No. 1

Email: kulikovaEA14@zdrav.mos.ru
ORCID iD: 0000-0002-0319-4934
SPIN-code: 2884-4803
Russian Federation, Moscow

Andrey L. Yudin

The Russian National Research Medical University named after N.I. Pirogov

Email: prof_yudin@mail.ru
ORCID iD: 0000-0002-0310-0889
SPIN-code: 6184-8284

MD, Dr. Sci. (Medicine), Professor

Russian Federation, Moscow

References

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Supplementary files

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2. Fig. 1. Lung fluorogram. A decrease in the volume of the right lung was revealed, a shading area without clear contours in the lower parts of the right pulmonary field (white arrow).

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3. Fig. 2. Computed tomograms of the lungs: a, b — axial plane; c — sagittal plane; d — coronal plane. The right lung is reduced in volume. The white arrows are a thickened interlobular interstitium; the yellow arrows are a cyst in the lung; the green arrow is a soft—tissue conglomerate of homogeneous structure, with a density of +40 HU in the mediastinum. When performing dynamic control using computed tomography from 2023, the picture was preserved without dynamics.

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4. Fig. 3. Computed tomograms of the lungs: a, b — axial plane; c — coronal plane. White arrows — multiple vascular collaterals along the contour of the bronchi; black arrow — hypoplasia of the right pulmonary artery.

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5. Fig. 4. Absence of right pulmonary veins (white arrows): a — computed tomography of the lungs in the coronal plane; b — computed tomography of the lungs in the axial plane; c — 3D reconstruction of the heart.

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