Centers of Excellence

The Lung Center

Pulmonary Vascular Disease

Pulmonary vascular disease (PVD) is a broad term including any condition that affects the blood vessels within the lungs. These vessels take blood that is depleted of oxygen to the lungs from the right side of the heart. Deoxygenated blood travels through the pulmonary arteries where oxygen is taken up. The pulmonary veins leave the lungs and take blood rich with oxygen to the left side of the heart where oxygenated blood is distributed throughout the body. This process continually replenishes the blood with oxygen, and lets carbon dioxide be exhaled. A pulmonary vascular disorder can lead to cardiovascular problems as well as impairing the quality of the patient’s life.

The Pulmonary Vascular Disease Program—a collaborative venture between the Brigham and Women’s Hospital Lung Center and the Heart & Vascular Center—offers personalized care and coordinated management for all types of PVD, including pulmonary arterial hypertension, right heart failure, and chronic thromboembolic pulmonary hypertension (CTEPH). Our pulmonologists and cardiologists work closely together. This expertise and collaboration helps patients manage symptoms and have an improved quality of life. The research on PVD which is conducted at BWH provides greater understanding of these diseases and is translated directly into exceptional patient care.

Pulmonary Vascular Disease (PVD) Topics

Pulmonary Hypertension

Pulmonary hypertension (PH) is high blood pressure in the blood vessels of the lungs. It occurs when the small blood vessels that go through the lungs become thicker, constrict tighter or become plugged. This leads to increased pressure in those vessels making the heart work harder to pump blood. If high pressure remains, the heart will become enlarged and weaker, pumping blood less efficiently into the lungs and eventually throughout the body. Types of PH include:

  • Pulmonary Arterial Hypertension Increased blood pressure in the pulmonary arteries (carrying blood away from the right side of the heart to the lungs). Pulmonary arterial hypertension can be caused by heart failure, lung disease or an autoimmune disease. If there is no apparent cause, it is called idiopathic pulmonary arterial hypertension.
  • Pulmonary Venous Hypertension Increased blood pressure in the pulmonary veins (carrying blood away from the lungs to the left side of the heart). Pulmonary venous hypertension is usually caused by poorly controlled systemic hypertension and congestive heart failure. A damaged mitral or aortic valve in the heart (mitral or aortic valve stenosis or mitral regurgitation) may also contribute to pulmonary venous hypertension.
  • Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is a rare form of both pulmonary hypertension and blood clots that go to the lung. Our pulmonary vascular team has experience diagnosing CTEPH and in both the medical and surgical treatment of this condition. Some, but not all, patients have a history of blood clots to the lung. Correct diagnosis is important because in many cases a surgical procedure called pulmonary thromboendarterectomy can successfully treat this condition. This procedure may be recommended for people whose pulmonary hypertension is due to persistent pulmonary emboli (blood clots).

Symptoms of Pulmonary Hypertension

  • Progressive fatigue
  • Shortness of breath
  • Chest pain or pressure during activity (angina)
  • Dizzy spells that may occur during activity or exercise
  • Fainting
  • Ankle or leg swelling (edema)
  • Increased heart rate (tachycardia)
  • Heart palpitations
  • Cough

Treatment for Pulmonary Hypertension

A team of BWH clinicians collaborate to treat each patient's pulmonary hypertension and associated medical conditions. Numerous treatment options are available, depending on the type of pulmonary hypertension.

Treatment for PH can lower patients' pulmonary pressure, reduce symptoms, increase exercise capacity, and prolong life expectancy.

Learn more about our advanced surgical, interventional, and medical CTEPH treatments.

Read the Pulmonary Advances Newsletter article: Improving Outcomes for Patients with Pulmonary Embolism and Chronic Thromboembolic Pulmonary Hypertension

Learn more about pulmonary hypertension.

Pulmonary Embolism

A pulmonary embolism occurs when a blood clot breaks off from a deep vein (usually in the leg), travels through the right side of the heart, and is pumped into the lungs.

An embolism to the lung may cause serious life-threatening consequences and, potentially, death.

Symptoms of Pulmonary Embolism:

  • Sudden shortness of breath (most common)
  • Chest pain (usually worse with breathing)
  • A feeling of anxiety
  • A feeling of dizziness, lightheadedness, or fainting
  • Palpitations (heart racing)
  • Coughing up blood (hemoptysis)
  • Sweating
  • Low blood pressure
  • Symptoms of deep vein thrombosis, such as:
    • pain in the affected leg (may occur only when standing or walking)
    • swelling in the leg
    • soreness, tenderness, redness, and/or warmth in the leg(s)
    • redness and/or discolored skin

Treatment for Pulmonary Embolism:

  • Anticoagulants also described as "blood thinners," these medications decrease the ability of the blood to clot. Examples of anticoagulants include warfarin (Coumadin®) and heparin.
  • Fibrinolytic therapy, also called "clot busters," these medications are given intravenously (IV) to break down the clot.
  • Vena cava filter, a small metal device placed in the vena cava (the large blood vessel that returns blood from the body to the heart) may be used to prevent clots from traveling to the lung. These filters are generally used in patients who cannot receive anticoagulation treatment (for medical reasons), who develop additional clots even with anticoagulation treatment, or who develop bleeding complications from anticoagulation. Some filters can be removed after a short period if the risk of future DVT and PE is reduced.
  • Pulmonary embolectomy is surgical removal of a pulmonary embolism. This procedure is generally performed only in severe situations in which the PE is very large, the patient either cannot receive anticoagulation and/or thrombolytic therapy due to other medical considerations or has not responded adequately to those treatments, and the patient's condition is unstable.
  • Percutaneous thrombectomy is the insertion of a catheter (long, thin, hollow tube) to the site of the embolism, using X-ray guidance. Once the catheter is in place, the catheter is used to break up the embolism, extract it (pull it out), or dissolve it by injecting thrombolytic medication.
Read more about pulmonary embolism.
Hepatopulmonary Syndrome

Hepatopulmonary syndrome is manifested by shortness of breath and hypoxemia (low oxygen levels in the blood of the arteries) caused by vasodilation (broadening of the blood vessels) in the lungs of patients with liver disease. The syndrome is most often found in patients with chronic and acute liver failure. The mechanism is unknown but may be due to increased liver production or decreased liver clearance of vasodilators, possibly involving nitric oxide.

Symptoms of Hepatopulmonary Syndrome:

  • Chronic or acute liver failure
  • Dyspnea (shortness of breath)
  • Low oxygen saturation

Treatment for Hepatopulmonary Syndrome:

The only definitive treatment is liver transplantation.

Causes of Pulmonary Vascular Disease

The causes of pulmonary vascular disease vary according to which of the lungs’ blood vessels are affected.

Diagnosis of Pulmonary Vascular Disease

In addition to taking a full medical history, your physician will conduct a variety of tests to diagnose PVD, as well as determine the specific disorder. These tests can include:

Treatment for Pulmonary Vascular Disease

In treating PVD, your physician aims to lessen the severity of symptoms, which will help improve your quality of life. This will also lessen the strain on your heart and decrease your risk for heart failure. You and your healthcare team will develop a personalized plan (based on your specific condition, age, and other factors) that may include a variety of medications and surgery.

What You Should Expect

When you become a patient, our team of experts develops a personalized, multidisciplinary care plan based on your specific condition. Our Pulmonary Vascular Disease Program provides long-term care and support to help patients manage their disease. We emphasize patient education, give patients access to research opportunities and offer a support group for patients and families to share personal experiences.

Multidisciplinary Care

The Pulmonary Vascular Disease Program—joint venture between the Brigham and Women’s Hospital Lung Center and the Heart & Vascular Center—offers highly specialized, multidisciplinary evaluation and care for patients with complex pulmonary vascular conditions. Our pulmonologists and cardiologists, work closely together and with cardiac and thoracic surgeons, and other cardiovascular specialists.


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