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 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 some patients, the elevated pressure may be related to obstructive sleep apnea syndrome (OSAS), and can be reduced with the use of a CPAP, BIPAP (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 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.

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.

Medically Reviewed by a Doctor on 2/19/2015

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