BACK TOTOP Browse A-ZSearchBrowse A-ZABCDEFGHIJKLMNOPQRSTUVWXYZ0-9 E-mail FormEmail ResultsName:Email address:Recipients Name:Recipients address:Message: Print-FriendlyBookmarksbookmarks-menuPulmonary veno-occlusive diseasePulmonary vaso-occlusive diseasePulmonary veno-occlusive disease (PVOD) is a very rare disease. It leads to high blood pressure in the lung arteries (pulmonary hypertension).Pulmonary hypertensionPulmonary hypertension is high blood pressure in the arteries of the lungs. It makes the right side of the heart work harder than normal.ImageRead Article Now Book Mark Article Causes In most cases, the cause of PVOD is unknown. The high blood pressure occurs in the pulmonary arteries. These lung arteries are directly connected to the right side of the heart.The condition may be related to a viral infection. It may occur as a complication of certain diseases such as lupus, or bone marrow transplantation.LupusSystemic lupus erythematosus (SLE) is an autoimmune disease. In this disease, the immune system of the body mistakenly attacks healthy tissue. It c...ImageRead Article Now Book Mark Article Bone marrow transplantationA bone marrow transplant is a procedure to replace damaged or destroyed bone marrow with healthy bone marrow stem cells. Bone marrow is the soft, fat...ImageRead Article Now Book Mark Article The disorder is most common among children and young adults. As the disease gets worse, it causes:Narrowed pulmonary veins Pulmonary artery hypertension Congestion and swelling of the lungsPossible risk factors for PVOD include:Family history of the condition Smoking Exposure to substances like trichloroethylene or chemotherapy medicines ChemotherapyThe term chemotherapy is used to describe cancer-killing drugs. Chemotherapy may be used to:Cure the cancer Shrink the cancerPrevent the cancer from...ImageRead Article Now Book Mark Article Systemic sclerosis (autoimmune skin disorder)Systemic sclerosisScleroderma is a disease that involves the buildup of scar-like tissue in the skin and elsewhere in the body. It also damages the cells that line th...ImageRead Article Now Book Mark Article Symptoms Symptoms may include any of the following:Shortness of breath Shortness of breathBreathing difficulty may involve:Difficult breathing Uncomfortable breathingFeeling like you are not getting enough airImageRead Article Now Book Mark Article Dry cough Fatigue on exertion Fatigue on exertionFatigue is a feeling of weariness, tiredness, or lack of energy.Read Article Now Book Mark Article Fainting FaintingFainting is a brief loss of consciousness due to a drop in blood flow to the brain. The episode most often lasts less than a couple of minutes and y...Read Article Now Book Mark Article Coughing up blood Coughing up bloodCoughing up blood is the spitting up of blood or bloody mucus from the lungs and throat (respiratory tract). Hemoptysis is the medical term for cough...Read Article Now Book Mark Article Difficulty breathing while lying flatDifficulty breathing while lying flatBreathing difficulty while lying down is an abnormal condition in which a person has a problem breathing normally when lying flat. The head must be ...ImageRead Article Now Book Mark Article Exams and Tests The health care provider will examine you and ask about your medical history and symptoms.The exam may reveal: Increased pressure in the neck veins Clubbing of the fingers ClubbingClubbing is changes in the areas under and around the toenails and fingernails that occur with some disorders. The nails also show changes.ImageRead Article Now Book Mark Article Bluish coloration of the skin due to lack of oxygen (cyanosis) Bluish coloration of the skinA bluish color to the skin or mucous membrane is usually due to a lack of oxygen in the blood. The medical term is cyanosis.ImageRead Article Now Book Mark Article CyanosisA bluish color to the skin or mucous membrane is usually due to a lack of oxygen in the blood. The medical term is cyanosis.ImageRead Article Now Book Mark Article Swelling in the legs Your provider may hear abnormal heart sounds when listening to the chest and lungs with a stethoscope.Heart soundsA heart murmur is a blowing, whooshing, or rasping sound heard during a heartbeat. The sound is caused by turbulent (rough) blood flow through the h...ImageRead Article Now Book Mark Article The following tests may be done:Arterial blood gases Arterial blood gasesBlood gases are a measurement of how much oxygen and carbon dioxide are in your blood. They also determine the acidity (pH) of your blood.ImageRead Article Now Book Mark Article Blood oximetry Chest x-ray Chest x-rayA chest x-ray is an x-ray of the chest, lungs, heart, large arteries, ribs, and diaphragm.ImageRead Article Now Book Mark Article Chest CT Chest CTA chest CT (computed tomography) scan is an imaging method that uses x-rays to create cross-sectional pictures of the chest and upper abdomen....ImageRead Article Now Book Mark Article Cardiac catheterization Cardiac catheterizationCardiac catheterization involves passing a thin flexible tube (catheter) into the right or left side of the heart. The catheter is most often insert...ImageRead Article Now Book Mark Article Lung function tests Echocardiogram EchocardiogramAn echocardiogram is a test that uses sound waves to create pictures of the heart. The picture and information it produces is more detailed than a s...ImageRead Article Now Book Mark Article Lung biopsy Treatment There is currently no known effective medical treatment. However, the following medicines may be helpful for some people:Medicines that widen the blood vessels (vasodilators) Medicines that control the immune system response (such as azathioprine or steroids)A lung transplant may be needed.Lung transplantLung transplant is surgery to replace one or both diseased lungs with healthy lungs from a human donor.ImageRead Article Now Book Mark Article Outlook (Prognosis) The outcome is often very poor in infants, with a survival rate of just a few weeks. Survival in adults may be months to a few years. Possible Complications Complications of PVOD may include:Difficulty breathing that gets worse, including at night (sleep apnea) Difficulty breathingBreathing difficulty may involve:Difficult breathing Uncomfortable breathingFeeling like you are not getting enough airImageRead Article Now Book Mark Article Sleep apneaObstructive sleep apnea (OSA) is a problem in which your breathing pauses during sleep. This occurs because of narrowed or blocked airways.ImageRead Article Now Book Mark Article Pulmonary hypertension Right-sided heart failure (cor pulmonale)Cor pulmonaleCor pulmonale is a condition that causes the right side of the heart to fail. Long-term high blood pressure in the arteries of the lung and right ve...ImageRead Article Now Book Mark Article When to Contact a Medical Professional Call your provider if you have symptoms of this disorder.Open ReferencesReferencesChin K, Channick RN. Pulmonary hypertension. In: Broaddus VC, Mason RJ, Ernst JD, et al, eds. Murray and Nadel's Textbook of Respiratory Medicine. 6th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 58.Churg A, Wright JL. Pulmonary hypertension. In: Leslie KO, Wick MR, eds. Practical Pulmonary Pathology: A Diagnostic Approach. 3rd ed. Philadelphia, PA: Elsevier; 2018:chap 12.Mclaughlin VV, Humbert M. Pulmonary hypertension. In: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 11th ed. Philadelphia, PA: Elsevier; 2019:chap 85.AllVideoImagesTogRespiratory system - illustration Air is breathed in through the nasal passageways, travels through the trachea and bronchi to the lungs.Respiratory systemillustrationRespiratory system - illustration Air is breathed in through the nasal passageways, travels through the trachea and bronchi to the lungs.Respiratory systemillustrationRelated Information Pulmonary hypertension(Condition)Swelling(Symptoms)Breathing difficulty(Symptoms) Review Date: 5/27/2020 Reviewed By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. 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