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</div><div><span>Pulmonary veno-occlusive disease</span></div></div>
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</a><h1 class="with-also" itemprop="name">Pulmonary veno-occlusive disease</h1>
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</noscript></div><div class="main"><div id="ency_summary"><p>Pulmonary veno-occlusive disease (PVOD) is a very rare disease. It leads to high blood pressure in the lung arteries (<a test="test" href="./000112.htm">pulmonary hypertension</a>).</p></div><section><div class="section"><div class="section-header"><div class="section-title"><h2>Causes</h2></div><div class="section-button"><button type="submit" aria-controls="section-1" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-1"><p>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.</p><p>The condition may be related to a viral infection. It may occur as a complication of certain diseases such as <a test="test" href="./000435.htm">lupus</a>, or <a test="test" href="./003009.htm">bone marrow transplantation</a>.</p><p>The disorder is most common among children and young adults. As the disease gets worse, it causes:</p><ul><li>Narrowed pulmonary veins</li><li>Pulmonary artery hypertension</li><li>Congestion and swelling of the lungs</li></ul><p>Possible risk factors for PVOD include:</p><ul><li>Family history of the condition</li><li>Smoking</li><li>Exposure to substances like trichloroethylene or <a test="test" href="./002324.htm">chemotherapy</a> medicines</li><li><a test="test" href="./000429.htm">Systemic sclerosis</a> (autoimmune skin disorder)</li></ul></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>Symptoms</h2></div><div class="section-button"><button type="submit" aria-controls="section-2" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-2"><p>Symptoms may include any of the following:</p><ul><li><a test="test" href="./003075.htm">Shortness of breath</a></li><li>Dry cough</li><li><a test="test" href="./003088.htm">Fatigue</a> on exertion</li><li><a test="test" href="./003092.htm">Fainting</a></li><li><a test="test" href="./003073.htm">Coughing up blood</a></li><li><a test="test" href="./003076.htm">Difficulty breathing while lying flat</a></li></ul></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>Exams and Tests</h2></div><div class="section-button"><button type="submit" aria-controls="section-3" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-3"><p>Your health care provider will examine you and ask about your medical history and symptoms.</p><p>The exam may reveal:</p><ul><li>
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Increased pressure in the neck veins
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</li><li><a test="test" href="./003282.htm">Clubbing</a> of the fingers
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</li><li><a test="test" href="./003215.htm">Bluish coloration of the skin</a> due to lack of oxygen (cyanosis)</li><li>
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Swelling in the legs
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</li></ul><p>Your provider may hear abnormal <a test="test" href="./003266.htm">heart sounds</a> when listening to the chest and lungs with a stethoscope.</p><p>The following tests may be done:</p><ul><li><a test="test" href="./003855.htm">Arterial blood gases</a></li><li>Blood oximetry</li><li><a test="test" href="./003804.htm">Chest x-ray</a></li><li><a test="test" href="./003788.htm">Chest CT</a></li><li><a test="test" href="./003419.htm">Cardiac catheterization</a></li><li><a test="test" href="./003853.htm">Lung function tests</a></li><li><a test="test" href="./003869.htm">Echocardiogram</a></li><li>Lung biopsy </li></ul></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>Treatment</h2></div><div class="section-button"><button type="submit" aria-controls="section-4" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-4"><p>There is currently no known effective medical treatment. However, the following medicines may be helpful for some people:</p><ul><li>Medicines that widen the blood vessels (vasodilators)</li><li>Medicines that control the immune system response (such as azathioprine or steroids)</li></ul><p>A <a test="test" href="./003010.htm">lung transplant</a> may be needed.</p></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>Outlook (Prognosis)</h2></div><div class="section-button"><button type="submit" aria-controls="section-5" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-5"><p>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. </p></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>Possible Complications</h2></div><div class="section-button"><button type="submit" aria-controls="section-6" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-6"><p>Complications of PVOD may include:</p><ul><li><a test="test" href="./003075.htm">Difficulty breathing</a> that gets worse, including at night (<a test="test" href="./000811.htm">sleep apnea</a>)</li><li>Pulmonary hypertension</li><li>Right-sided heart failure (<a test="test" href="./000129.htm">cor pulmonale</a>)</li></ul></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>When to Contact a Medical Professional</h2></div><div class="section-button"><button type="submit" aria-controls="section-7" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-7"><p>Contact your provider if you have symptoms of this disorder.</p></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>Alternative Names</h2></div><div class="section-button"><button type="submit" aria-controls="section-Alt" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-Alt"><p>Pulmonary vaso-occlusive disease</p></div></div></section><section><div class="section sec-mb"><div class="section-header"><div class="section-title"><h2>Images</h2></div><div class="section-button"><button type="submit" aria-controls="section-tnails" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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<li><img src="//medlineplus.gov/ency/images/ency/tnails/9248t.jpg" alt="Respiratory system" title="Respiratory system" class="side-img"/><a href="../imagepages/9248.htm">Respiratory system</a></li>
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</ul></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>References</h2></div><div class="section-button"><button type="submit" aria-controls="section-Ref" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-Ref"><p>Churg A, Wright JL. Pulmonary hypertension. In: Smith ML, Leslie KO, Wick MR, eds. <em>Practical Pulmonary Pathology: A Diagnostic Approach</em>. 4th ed. Philadelphia, PA: Elsevier; 2024:chap 12.</p><p>Lammi MR, Mathai SC. Pulmonary hypertension: general approach. In: Broaddus VC, Ernst JD, King TE, et al, eds. <em>Murray and Nadel's Textbook of Respiratory Medicine</em>. 7th ed. Philadelphia, PA: Elsevier; 2022:chap 83.</p><p>Maron BA. Pulmonary hypertension. In: Libby P, Bonow RO, Mann DL, Tomaselli GF, Bhatt DL, Solomon SD, eds. <em>Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine</em>. 12th ed. Philadelphia, PA: Elsevier; 2022:chap 88.</p></div></div></section>
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<section><div class="section"><div class="section-header"><div class="section-title"><h2>Review Date 6/17/2024</h2></div><div class="section-button"><button type="submit" aria-controls="section-version" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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