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Medical Author:

George Schiffman, MD, FCCP

George Schiffman, MD, FCCP

Dr. Schiffman received his B.S. degree with High Honors in biology from Hobart College in 1976. He then moved to Chicago where he studied biochemistry at the University of Illinois, Chicago Circle. He attended Rush Medical College where he received his M.D. degree in 1982 and was elected to the Alpha Omega Alpha Medical Honor Society. He completed his Internal Medicine internship and residency at the University of California, Irvine.

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Medical Author:

Siamak T. Nabili, MD, MPH

Siamak T. Nabili, MD, MPH

Dr. Nabili received his undergraduate degree from the University of California, San Diego (UCSD), majoring in chemistry and biochemistry. He then completed his graduate degree at the University of California, Los Angeles (UCLA). His graduate training included a specialized fellowship in public health where his research focused on environmental health and health-care delivery and management.

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Medical Editor:

William C. Shiel Jr., MD, FACP, FACR

William C. Shiel Jr., MD, FACP, FACR

Dr. Shiel received a Bachelor of Science degree with honors from the University of Notre Dame. There he was involved in research in radiation biology and received the Huisking Scholarship. After graduating from St. Louis University School of Medicine, he completed his Internal Medicine residency and Rheumatology fellowship at the University of California, Irvine. He is board-certified in Internal Medicine and Rheumatology.

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In this Article

• What are pulmonary arteries?
• What is pulmonary hypertension?
• What are primary and secondary pulmonary hypertension?
• What causes pulmonary hypertension?
• What causes primary pulmonary hypertension?
• How common is pulmonary hypertension?
• What are the signs and symptoms of pulmonary hypertension?
• How is pulmonary hypertension diagnosed?
• What is the treatment for pulmonary hypertension?
• What is the life expectancy for pulmonary hypertension?
• Patient Comments: Pulmonary Hypertension - Share Your Experience
• Patient Comments: Pulmonary Hypertension - Treatments
• Patient Comments: Pulmonary Hypertension - Symptoms
• Find a local Cardiologist in your town
• Pulmonary Hypertension Index

The treatment for pulmonary hypertension depends on the underlying cause.

If left sided heart failure is the primary problem, then adequate treatment of the left heart failure by a cardiologist is the main stray of treatment.

In cases where hypoxia (low oxygen levels) due to any chronic lung disease, such as COPD, is the cause, then providing oxygen and appropriately treating the underlying lung disease by a lung doctor (pulmonologist) is the first step in treatment.

In conditions, such as scleroderma, which often can cause pulmonary hypertension, a rheumatologist is involved in the treatment program.

Anticoagulation (thinning the blood) may be a treatment option if the main underlying cause is thought to be recurrent blood clot (chronic thromboembolic pulmonary hypertension). As indicated in previous section, referral to a specialty center may be warranted for a possible surgical removal of blood clot (thromboendarterectomy).

For patients with primary pulmonary hypertension (those with no underlying cause), more advanced therapy may be attempted. These drugs have complex mechanisms, but in general they work by dilating (opening up) the pulmonary arteries and, therefore, by reducing the pressure in these blood vessels and some help prevent the excessive overgrowth of tissue in the blood vessels (that decrease remodeling of the vessels, as described above).

There are three major classes of drugs used to treat idiopathic pulmonary hypertension and pulmonary hypertension associated with collagen vascular diseases: 1) prostaglandins; 2) phosphodiesterase type 5 inhibitor; and 3) endothelium antagonists.

1. Prostaglandins such as epoprostenol (Flolan), treprostinil (Remodulin), iloprost (Ventavis). These drugs are very short-acting and often must be given intravenously or inhaled on a very frequent or continuous basis.
   
   
2. Phosphodiesterase type 5 inhibitors such as sildenafil (Revatio, Viagra) and tadalafil (Adcirca, Cialis) are somewhat less effective than the prostaglandins, but are easily administered one to three times per day by mouth. (The dosing is much different when these drugs are used for erectile dysfunction.)
   
   
3. Endothelium antagonists are the newest medications used for this condition. These include bosentan (Tracleer) and Ambrisentan (Letairis). These medications are also given by mouth one to two times per day.

In rare cases, calcium channel blockers may be of benefit.

Currently, research is investigating the best ways to combine these medications for the optimal clinical outcomes.

It should be noted that these medications are extremely expensive, costing thousands of thousands of dollars per year. The companies that manufacture these medications often have programs to assist in funding.

These more advanced therapies have also been used for other forms of pulmonary hypertension, however, no clinical studies have yet confirmed benefits in these situations.

It is worth mentioning that regardless of the cause of pulmonary hypertension, supplemental oxygen and diuretics (water pills) may play an important role in relieving the symptoms of pulmonary hypertension of any cause. Low oxygen in the atmosphere causes low blood oxygen levels and aggravates pulmonary hypertension. Therefore, patients with pulmonary hypertension may benefit from breathing supplemental oxygen, especially during air travel or traveling to high altitude destinations.

Despite advances in various treatments, there is no cure for pulmonary hypertension.