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Pulmonary Hypertension - Pulmonary Vascular Disease Program

What Is 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, in turn, leads to increased stiffness and pressure in those vessels making the right side of the heart work harder to pump blood through the lungs. If high pressure remains, the heart will become enlarged and weaker, pumping blood less efficiently into the lungs and eventually throughout the body. Patients develop progressive fatigue and shortness of breath, two typical symptoms of PH.

Given the complexity of some of the drugs and how they are given, it is very important that physicians who can diagnose and treat PH with the full range of treatment options evaluate patients at a pulmonary hypertension center.

What are the symptoms of pulmonary hypertension?

Early in the disease, symptoms may be nonspecific and can mimic symptoms of other medical conditions. It is also possible that patients may experience only limited symptoms.

The following are some of the most common symptoms of PH:

  • Worsening shortness of breath (also called dyspnea)
  • Fatigue or tiredness
  • 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 throbbing (palpitations)
  • Cough

What causes pulmonary hypertension?

While we do not yet know the causes of PH, we do know that it affects people of all ages, races and ethnic backgrounds. There are five categories of PH based on either common changes in patient's blood vessels and heart function or associated diseases. Patients with connective tissue disease, liver disease, and HIV disease are at risk for Pulmonary Arterial Hypertension or PAH. Likewise, patients with valvular or hypertensive heart disease, or severe lung disease can also develop different forms of PH. We have an active research program at the Brigham and Women's Hospital looking into the causes of all forms of PH.

Treatment of Pulmonary Arterial Hypertension

At the Brigham and Women's Hospital a team of clinicians who collaborate to treat each patient's pulmonary hypertension and associated medical conditions cares for patients. We are experienced in treating all forms of pulmonary hypertension. Numerous treatment options for pulmonary hypertension are available, depending on the type of pulmonary hypertension.

Treatment for PH can lower patients' pulmonary pressure, reduce symptoms, increase exercise tolerance, and prolong life expectancy. Treatments for pulmonary arterial hypertension range from medications to transplant surgery. Our researchers are also studying new treatments for PAH all the time.

Medications

Several types of medications are used to treat PAH.

  • Prostacyclin analogues facilitate the vessels in the lungs to open more and allow blood to move through them with less resistance (vasodilation). These drugs may be given by continuous IV infusion, infusion under the skin, or as an inhaled therapy. We are exploring other delivery methods for prostanoids, including oral administration.
  • Endothelin receptor antagonists, in pill form, help reverse the effect of endothelin, a substance in blood vessels that causes constriction.
  • Phosphodiesterase-5 inhibitors relax the blood vessels in the lungs.
  • Anticoagulants help prevent the blood from clotting.
  • Diuretics help the body remove excess fluid that may accumulate in tissues due to high pressures in blood vessels.
  • Disease Modifying and Antiproliferative agents are new drugs that may have a role in treatment of PAH by reducing abnormal growth of the cells that line the lung arteries.

Many drugs for treating pulmonary hypertension have side effects. The dosage level must be carefully set and monitored to avoid serious side effects or complications. We work with patients to find the most effective and safe doses.

Chronic Thromboembolic Pulmonary Hypertension

Chronic thromboembolic disease (CTED) and chronic thromboembolic pulmonary hypertension (CTEPH) are rare form of pulmonary vascular disease and pulmonary hypertension that occurs after blood clots go to the lung, called pulmonary embolism or PE. Our pulmonary vascular team has experience in diagnosing and the medical and surgical treatment of this condition. Importantly, not all patients have a recognized history of blood clots to the lung. Correct diagnosis is important because in many cases a surgical procedure called pulmonary thromboendarterectomy (PTE) can successfully treat this condition. This procedure may be recommended for people whose pulmonary hypertension is due to persistent pulmonary emboli (blood clots).

Estimates of the incidence of CTEPH suggest that up to 4% of patients surviving acute PE will develop the disease. In the United States, more than 500,000 individuals suffer an acute pulmonary embolism while the annual number of new CTEPH cases in the United States is between 500 and 2,500. This is probably an underestimate of the true frequency of CTEPH since the disease is often misdiagnosed due to nonspecific symptoms and variable course.

In patients with chronic thromboembolic pulmonary hypertension (CTEPH), pulmonary thromboendarterectomy (PTE) is potentially curative therapy. However, some patients are not suitable candidates for PTE, have persistent pulmonary hypertension (PH) after PTE, or need treatment before PTE. In such patients, PH-therapy is often administered

For patients who may not be eligible for surgery or who have recurrent or persistent pulmonary hypertension after surgery, medical treatment is beneficial. In addition, percutaneous balloon pulmonary angioplasty (BPA) is an emerging option and promises hemodynamic and functional benefits for inoperable patients.

Follow-Up Care

We not only diagnose and treat pulmonary hypertension, we also provide long-term care and support to help patients manage their condition. Our team of physicians and nurses develop individualized treatment plans and follow up with each patient on a regular schedule. Clinicians are available to answer patients' questions as needed. Patients also can access information about research opportunities and continuing education. Close follow-up care helps physicians detect early changes that may require further treatment.

Participation in Clinical Trials

Our program participates in a large number of industry sponsored and investigator initiated clinical trials and research studies. Clinical trials are designed to find new or better ways to treat, prevent or diagnose a medical condition. Clinical trials involve human participants to evaluate the safety and effectiveness of new approaches to, and treatments for, diseases like pulmonary hypertension.

In clinical trials, patients receive specific interventions according to a research plan, such as new drugs or devices. We are always exploring different combinations of therapies, whether it be introducing a new drug or using a standard (approved) treatment in a new way to find the best and most effective approach to treating patients. There are several benefits of participating in a clinical trial:

  • You will get access to the newest treatments at no cost to you or your insurance company
  • You might receive access to the latest treatment options before they become available.
  • You are very carefully monitored by health care professionals every step of the way
  • You are participating in research that could bring medical advances to the treatment of pulmonary hypertension
  • If you would like to hear more about participating in a clinical trial you can talk with your provider or reach out to our offices. Email: BWHPVDProgram@Partners.org

Useful Links

Pulmonary Hypertension Association

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