Concise Overview of Pulmonary Vein Stenosis

The right side of the heart normally pumps blood to the lung. The left side of the heart normally pumps blood to the body. The blood normally becomes full of oxygen as it passes through the lung. The pulmonary veins are the blood vessels that return oxygenated blood from the lung to the left side of the heart. Two to three pulmonary veins from the right side of the lung and two pulmonary veins from the left side of the lung usually connect with the left atrium. Occasionally, the right or left veins will join before entering the left atrium and form a common or single right-sided or left-sided connection.

During early fetal development, anomalous pulmonary venous connections may form, and oxygenated blood may return to the right side of the heart instead of the left side of the heart. The terms total anomalous pulmonary venous connection, drainage or return are used when all pulmonary veins ultimately drain to the right side of the heart. The terms partial anomalous pulmonary venous connection, drainage or return are used when only some of the pulmonary veins drain to the right side of the heart. Anomalous veins are usually detected during infancy or childhood and can usually be corrected with surgery.

Pulmonary venous connections may become narrow and impede the return of oxygenated blood from the lung to the left side of the heart. Narrowing may occur with normal pulmonary venous connections or anomalous pulmonary venous connections after surgical repair. The cause of narrowing is often unknown. External compression, inflammation and genetic variants are some of the factors that potentially influence the onset and severity of narrowing in normal venous connections. A suboptimal surgical anastomosis or intrinsic abnormalities in the vessel wall may lead to narrowing of the pulmonary veins after repair of anomalous connections. In either case, a process develops inside the vessel wall (intraluminal), which may progress from the veins near their connection with the heart (extraparenchymal) upstream into the veins of the lung tissue (parenchyma). A link under Additional Resources will direct you to an excellent overview of intraluminal pulmonary vein stenosis with illustrations on the Pulmonary Vein Stenosis Network website.

Pulmonary veins may appear to have no connection with the left or right side of the heart. The term pulmonary vein atresia is used when there is no apparent connection with the heart. Though it is difficult to know whether the connection failed to develop, or a narrow vein became progressively more obstructed over time.

Click here for an illustration that may be used to classify the severity of intraluminal stenosis of individual pulmonary veins.

The severity of pulmonary venous narrowing that occurs with normal connections and anomalous connections after surgical repair is different in individual patients. The spectrum of disease may vary from mild static narrowing of one or two pulmonary veins returning from one side of the lung to severe progressive narrowing of multiple veins returning from both sides of the lungs. Patients with mild static narrowing of one or two veins may have minimal symptoms, while patients with severe progressive narrowing of multiple veins may develop congestion in the lungs, labored breathing, right-sided heart failure and death.

Purpose of the Archive

Our understanding of pulmonary venous obstruction is improving. However, we need to learn much more about the disease process, diagnostic imaging, medical therapy, and optimal interventions to improve the care of affected patients. The Pediatric Pulmonary Vein Stenosis Archive, pvsarchive.org, was created to provide a collection of nearly all the articles that have been published concerning intraluminal (inside the vessel) narrowing or obstruction of the extraparenchymal (between the lung and heart) pulmonary veins in children. The archive will hopefully be a valuable resource for care providers to readily identify what is known and what is not known about this rare disease. Learn more from the publications by searching individual categories, keywords, or a year of publication.

The archive does not list all publications related to pulmonary venous obstruction. The following subsets of disease have not been included in order to focus primarily on an intraluminal process that initially develops in the extraparenchymal pulmonary veins:

•   Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis: These conditions affect very small veins within the lung. The extraparenchymal pulmonary veins are usually normal.

•   Pulmonary venous obstruction in adults: Catheter-mediated interventions for atrial fibrillation, malignancy, and mediastinal inflammation or infection are leading causes of pulmonary venous obstruction in adults. Most publications related to these causes of disease have not been included in this archive. However, publications concerning adults with pulmonary vein stenosis or atresia have been included if their disease process likely developed before birth or when they were children.

•   Obstruction of anomalous pulmonary venous connections before surgical repair: Anomalous pulmonary venous connections may be compressed or obstructed before they join with the heart. Of note, publications that identified preoperative obstruction as a risk factor for postoperative intraluminal obstruction have been included.

•   Focal obstruction of the pulmonary venous anastomosis site alone following surgical repair of anomalous pulmonary venous connections

•   Focal obstruction of pulmonary venous flow through a narrow atrial baffle site alone.

•   The archive does not include publications that were not identified by searching PubMed or references within the publications of this archive.

Please help improve the scope and accuracy of the Pulmonary Vein Stenosis Archive. Click on “Contact” in the header of this page and send a message with recommendations for additional publications to include, or errors to correct.

Additional Resources

Pulmonary Vein Stenosis Network
https://pvsnetwork.org

Pediatric Pulmonary Hypertension Network
https://pphnet.org

Pulmonary Hypertension Association
https://phassociation.org

Instructions for Searching the Content of this Website

Category

Simply click on the category in the side bar on this page.

Search Buttons

Both search buttons in the header of this page can be used to search a single keyword, name or year. 

Search a Single Year of Publication

Enter the year followed by a semicolon (eg. 2020;) in either search button. Click here to see a graph with the years that articles were published concerning intraluminal pulmonary vein stenosis or atresia on this site.

Search: “and” Function

Select this search function in the header to identify only the posts that contain all keywords, names and years that are entered. If an author’s name and a year are entered (eg. Jenkins 2021;), only the publications by the author during that year will be displayed. If more than one year is entered (eg. 2021; 2022;), there should be no results.

Search: “or” Function

Select this search function in the header to identify the posts that contain any of the keywords, names and years that are entered. This search function can be used to identify articles from more than one year.  If more than one year is entered (eg. 2021; 2022; 2023;), all of the publications during those years will be displayed. If an author’s name and a year is entered (eg. Vanderlaan 2021;), publications by the author from all years, and all of the publications by other authors during that year will be displayed. 

Article Access

Each post indicates whether there is free access to a PDF File or Full Text Article through PubMed or the publication’s DOI. Access can usually be made by clicking on the DOI in the post, or searching the reference in PubMed.