Pulmonary Hypertension

  • Medical Author:
    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.

  • Medical Editor: William C. Shiel Jr., MD, FACP, FACR
    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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What medications treat pulmonary hypertension?

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-receptor antagonists.

  1. Prostaglandins such as epoprostenol (Flolan), treprostinil (Remodulin, Tyvaso), 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.

What other drugs treat pulmonary hypertension?

  • A unique drug, riociguat (Adempas), that is indicated for pulmonary hypertension due to chronic thromboembolic disease (CTEPH). The mechanism of action is different then the drugs above. It works by increasing the effect of nitric oxide causing increased pulmonary vasodilation.
  • In rare cases, calcium channel blockers (CCBS) 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.

Medically Reviewed by a Doctor on 3/30/2016

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