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Pulmonary Hypertension (cont.)

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What is the treatment for pulmonary hypertension?

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 mainstay 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 some patients, the elevated pressure may be related to obstructive sleep apnea syndrome (OSAS), and can be reduced with the use of a CPAP, (a device that delivers positive airway pressure during sleep).
  • 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 previously). Besides constriction, PAH can result in an actual structural change of the pulmonary arteries, whether it is due to chronically elevated pressures or the disease itself, or a combination of the two is not completely clear. These drugs also can delay and in some cases reduce the degree of remodeling of the pulmonary arteries.


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 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.

Is there a cure?

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

What is the life expectancy?

Generally, the prognosis of pulmonary varies depending on the underlying condition that is causing it. For idiopathic or familial pulmonary hypertension, the overall prognosis depends on the severity and whether treatment was instituted. The statistics show a survival of about 3 years in idiopathic pulmonary hypertension without any therapy. Some of the other factors may indicate even poorer prognosis which include severe symptoms, age of onset greater than 45 years, evidence of right sided heart failure, and failure to respond to treatment. For patients with idiopathic pulmonary hypertension who get started on treatment and respond to it, the prognosis is better. Studies are underway to determine optimal treatment regimens.

Which types of doctors treat pulmonary hypertension?

Generally, pulmonary physicians (pulmonologists) and cardiologists treat pulmonary hypertension. Depending on the cause, other physicians also may be involved such as sleep medicine doctors, rheumatologists, and perhaps even infectious disease specialists. Today, more health-care professionals in the community are becoming adept at managing pulmonary hypertension. However, it is reasonable to get a second opinion regarding management at a tertiary, university setting because the field of management of pulmonary artery hypertension is evolving rapidly.


Kasper, D.L., et al., eds. Harrison's Principles of Internal Medicine, 19th Ed. United States: McGraw-Hill Education, 2015.

Pom and Kingman; Inhaled treprostinil for pulmonary arterial hypertension. CCN.2009;31(6);e1-e11.

Kiely, et al. Pulmonary hypertension;diagnosis and management. BBMJ 2013;346:f2028

Raza, T and Dilawar, M; Pulmonary Hypertension; A Comprehensive Review; Heart Views Vol. 8 No.3 Sept-Nov 2007; 90-99

Simonneau, G, Galie, N, Rubin, LJ, et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol 2004; 43:5S.

Simonneau, et al. Updated Clinical Classification of Pulmonary Hypertension. J Am Coll Cardiol 2009;54:43-54, doi:10.1016/j.jacc.2009.04.012

Medically Reviewed by a Doctor on 7/26/2017


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