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What medical therapies are available for treating pulmonary vein stenosis?

There is no known medical therapy to cure pulmonary vein stenosis. This is an area of research that is of great interest. Medical therapies for pulmonary vien stenosis can be broken down into 2 different categories.
​1.  Therapies to treat the symptoms of pulmonary vein stenosis
​2. Experimental therapies to try to treat pulmonary vein stenosis. 

Therapies to treat the symptoms of pulmonary vein stenosis

Therapies that are commonly used to treat the symptoms of pulmonary vein stenosis are things like therapies to manage fluid,  to optimize heart function and sometimes to manage pulmonary hypertension.

​Diuretics are a class of medications that help regulate the amount of fluid in the body. Typically, if a child has severe pulmonary vein stenosis, the right heart is working harder to pump blood. A natural response in the body is to increase the amount of fluid in the body, but this can cause the right heart to work even harder. To decrease the amount of fluid that needs to be pumped, diuretics are used to help your child to regulate the amount of fluid. A common one used is Lasix or furosemide.

​Depending on the status of your child's heart and other congenital heart defects that are present, your child may be on medications to help optimize or protect the heart's function. These will be specfic to your child.

​Pulmonary hypertension is an important result of pulmonary vein stenosis. Sometimes, pulmonary hypertension medications can be used for a short time in the post-operative course or in special circumstances.  Pulmonary hypertension medications can open up the pulmonary arteries, but they can not relieve the narrowing or obstruction in the pulmonary veins and in severe circumstances they make things worse.  Your child's cardiologist will know if these medications are right for your child.

Experimental Therapies to treat pulmonary vein stenosis

There are several experimental therapies that are being tested. These are currently experimental and have not been proven to slow the progress of the disease yet. [ link to clinical trials page].

​Current experimental therapies are used to target the accumulation of cells that cause the blockage. Some of these cells can be from proliferation and some from a process that transforms cells ( called endothelial- to mesenchymal transition) into cells found in the lesions of pulmonary vein stenosis. 

If there is a clinical trial, your child may be eligible to be enrolled.  Talk to your cardiologist about if this is best for your child.
​

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  • Home
    • About PVS Network
    • Participating institutions
    • Creating Hope
  • Parents
    • About PVS
    • Clinical tests >
      • Echocardiography
      • CT Scan
      • MRI Scan
      • Heart catheterization
      • Lung perfusion scan
    • Treatment options >
      • Surgery
      • Catheter based intervention
      • Medical therapy
      • Lung transplant
      • Palliative care
    • Frequently asked questions
    • Links
  • Clinicians
    • PVS: outcomes and pathophysiology
    • Clinical Care for Children with PVS >
      • Treatment options >
        • Surgical Options
        • Catheter based interventions
        • Medical therapies
        • Transplantation
        • Palliative Care
      • Surveillance imaging
    • Clinical Scenarios: tough decisions
    • Clinical Trials and Research
  • Research
    • PVS Network Registry >
      • PVS Registry Image Guide
      • PVS data collection forms 2017
    • PVS Clinical Research
    • Recently Published
  • Contact Us
    • Ways to Donate
    • Links to Related Sites
    • Newsletters
  • PVS Awareness
    • T- shirt Campaign
    • Knowledge is Hope
    • TEAM PVS!
    • Resolution
    • Superhero
    • Donate Life Month
    • Supermom
    • Sibling
    • PVS Aware
    • Life is a beautiful ride