Pulmonary Hypertension

Treatment

At Mayo Clinic, a team of physicians collaborate to treat each patient who has pulmonary hypertension. Depending on the type and cause of pulmonary hypertension, treatment options may include medications and surgical procedures. Surgeons at Mayo Clinic are experts in performing complex surgeries to treat pulmonary hypertension, including lung transplants and pulmonary thromboendarterectomies. Mayo physicians also provide extensive follow-up and management care to patients after initial treatment.

Treatment of Pulmonary Arterial Hypertension

Pulmonary arterial hypertension (PAH) is a relatively rare disease that has no cure yet. However, treatment can lower patients' pulmonary pressure, reduce symptoms, increase the capacity for activity, and prolong lifespan. Treatments for pulmonary arterial hypertension range from medications to transplant surgery. Mayo Clinic researchers are also studying new treatments for PAH.

Transplant Surgery

In some cases, a lung or heart-lung transplant may be an option, at least for younger patients with PAH. Testing determines whether a patient is a good candidate for a lung or heart-lung transplant. Lung transplant surgery can eliminate pulmonary hypertension but requires extensive post-transplant monitoring and care. Specialists at Mayo Clinic have extensive experience in transplant surgery. Read more about transplant surgery.

Medications

Several types of medications are used to treat PAH.

• Prostacyclin analogues enable the vessels in the lungs to expand 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. Mayo Clinic researchers 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. Medications that may enhance the response of blood vessels to phosphodiesterase-5 inhibitors are being studied at the Mayo Clinic.
• High-dose calcium channel blockers may help relax the muscles in the blood vessels and promote vasodilation for some patients.
• 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.
• Antiproliferative agents are anti-cancer drugs that may have a role in treatment of PAH by reducing abnormal growth of the cells that line the lung arteries. Mayo researchers are participating in research trials examining the use of these drugs in treating PAH.

Many drugs for treating pulmonary hypertension have strong, serious side effects. The dosage level must be carefully set and monitored to avoid dangerous consequences. Mayo physicians work with patients to find the most effective doses.

Oxygen

With a higher concentration of oxygen in the air, more oxygen enters the bloodstream, and the constricting effects of low oxygen levels on lung vessels are reduced.

Treatment of Associated Pulmonary Arterial Hypertension

Because other conditions can cause pulmonary hypertension, the underlying disease is treated along with the blood vessel disease. Successful treatment of the underlying disease may decrease the severity of pulmonary hypertension. Mayo Clinic cardiologists work with a team of medical specialists to treat a patient's conditions. If the underlying disease cannot be cured, the pulmonary hypertension may be treated directly, using many or all of the therapies used to treat PAH.

Chronic Thromboembolic Pulmonary Hypertension

Chronic thromboembolic pulmonary hypertension is often missed or misdiagnosed. Physicians at Mayo Clinic have experience in diagnosing and treating this condition. Some, but not all, patients have a history of blood clots to the lung. Correct diagnosis is important because pulmonary thromboendarterectomy can successfully treat this condition. This procedure may be recommended for people whose secondary pulmonary hypertension is due to persistent pulmonary emboli (blood clots).

Follow-Up Care

At Mayo Clinic, physicians not only treat pulmonary hypertension, they also provide long-term care and support to help patients manage their condition. The team of physicians and nurses develop individualized treatment plans and follow up with each patient. Physicians are available to answer patients' questions by telephone, consulting other specialists 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.