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Case Reports
. 2020 Jun 8;8(9):1698-1703.
doi: 10.1002/ccr3.2982. eCollection 2020 Sep.

Left hypoplastic lung and hemoptysis-rare familial unilateral pulmonary vein atresia

Ronly Har-Even Cohn  1 , Matthew Hicks  1 , Atilano Lacson  2 , Anne Hicks  1
Affiliations
Case Reports

Left hypoplastic lung and hemoptysis-rare familial unilateral pulmonary vein atresia

Ronly Har-Even Cohn et al. Clin Case Rep. .

Abstract

Unilateral pulmonary vein atresia (UPVA) is a rare congenital vascular malformation with obliteration of the pulmonary vein. We present a case series of three siblings with variable presentation of UPVA. We suggest a dominant genetic cause based on different paternity. Identifying genetic etiology would contribute to early diagnosis and screening.

Keywords: pulmonary hypertension (PH); pulmonary vein stenosis (PVS); unilateral pulmonary vein atresia (UPVA).

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Conflict of interest statement

Dr Cohn, Dr A Hicks, Dr Lacson, and Dr M Hicks declare that they have no competing interests and did not receive any personal financial support.

Figures

Figure 1
Figure 1
CXR—L hypoplasia
Figure 2
Figure 2
CTA—abnormalities in lung parenchyma and vasculature. The pulmonary veins were not demonstrated near the LA
Figure 3
Figure 3
V/Q scan—severe decreased L ventilation and perfusion
Figure 4
Figure 4
Gross photograph of left lung with focal pleural fibrosis and consolidation of the upper lob. Encircled, ligated arterial collaterals
Figure 5
Figure 5
A low‐power view of left upper lobe stained with MOVAT to show consolidation with honeycombed fibrosis
Figure 6
Figure 6
CXR—bilateral opacities
Figure 7
Figure 7
CTA diffuse interstitial reticular markings with multifocal opacities
See this image and copyright information in PMC

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