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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Pulmonary vein stenosis


Other Names for this Disease
  • Pulmonary veins stenosis
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Overview


Pulmonary vein stenosis is a very rare and serious condition in which there is a blockage in the blood vessels that bring oxygen-rich blood from the lungs back to the heart. This condition can be isolated to one vein, but often affects multiple veins. Stenosis occurs when there is an abnormal thickening and narrowing of the walls of the veins. Pulmonary vein stenosis is a progressive condition and may lead to total obstruction to a blood vessel. Most commonly, all of the pulmonary veins of one lung are affected, causing pulmonary hypertension and pulmonary arterial hypertension. Surgery and catheterization to widen the narrow veins is usually a short-term solution since the obstruction typically recurs.[1][2]
Last updated: 9/1/2011

References

  1. Hövels-Gürich H. Congenital pulmonary veins stenosis. Orphanet. March 2003; http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=3459. Accessed 9/1/2011.
  2. About pulmonary vein stenosis. Children's Hospital Boston. 2011; http://www.childrenshospital.org/clinicalservices/Site683/mainpageS683P7.html. Accessed 9/1/2011.
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In Depth Information

  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Pulmonary vein stenosis. Click on the link to view a sample search on this topic.