Treatments for Pulmonary Hypertension

The primary treatment of pulmonary hypertension is to catch the process early and identify and treat the underlying cause. However, in many patients with this condition, more advanced therapy becomes necessary.

Baseline Assessment

The first step in treating pulmonary hypertension is to assess the baseline severity of the condition. This testing helps the doctor decide how aggressive to be with treatment, and provides a way to assess the response to therapy.

This is usually done by performing an echocardiogram, which will provide an estimate of the pulmonary artery pressure, and an exercise test to measure baseline functional capacity.

Treatment Aimed at the Underlying Medical Condition

Typically, aggressively treating the underlying cause of pulmonary hypertension is the most critical aspect of therapy. Since there are many medical disorders that can produce pulmonary hypertension, this therapy can take many different forms.

For instance, if pulmonary hypertension is caused by chronic pulmonary emboli, anticoagulation (blood thinners) should be given. If it is caused by sarcoidosis, anticoagulation should be withheld, and treatment aimed at the sarcoidosis (such as intravenous prostacyclin) should be considered.

Treatments Often Useful for Anyone With Pulmonary Hypertension

In addition to therapy aimed specifically at treating the underlying cause, there are treatments that are often helpful in anyone who has pulmonary hypertension.

These include:

• Diuretics such as Lasix or Bumex are often used to treat the fluid retention that frequently occurs with pulmonary hypertension. While diuretics can improve dyspnea and edema (swelling), they must be used carefully since getting rid of too much fluid in patients with pulmonary hypertension can worsen cardiac function.
• Oxygen therapy is important in anyone with pulmonary hypertension who has hypoxia (reduced blood oxygen levels). When hypoxia is present, administering oxygen can often directly reduce pulmonary artery pressures. Patients whose pulmonary hypertension is caused by lung diseases or cardiac diseases are especially likely to have hypoxia, but anyone with pulmonary hypertension should have their blood oxygen levels checked.
• Anticoagulants such as Coumadin are often used in patients with pulmonary hypertension. This is because the pulmonary hypertension itself can result in sluggish blood flow through the pulmonary circulation, which can lead to clotting within the blood vessels in the lungs. Read about drugs used to prevent blood clots.
• Digoxin can improve cardiac function in some patients with pulmonary hypertension.
• Exercise has been shown to significantly improve the functional capacity of people with pulmonary hypertension.
• Vaccination for influenza and pneumococcal pneumonia, as well as other age-appropriate vaccinations, are important to prevent infectious diseases that can worsen pulmonary hypertension.

Advanced Therapy

"Advanced therapy" for pulmonary hypertension is aimed at the pulmonary hypertension itself, rather than the underlying cause. This therapy generally consists of using vasodilator drugs to attempt to dilate the pulmonary circulation and reduce pulmonary artery pressures.

Advanced therapy is considered in patients with severe pulmonary hypertension that has not adequately responded to treatment aimed at the underlying cause, or, especially, if no underlying cause has been identified (that is, in people with primary pulmonary hypertension).

Advanced therapy is usually not used at all in people whose pulmonary hypertension is due to underlying heart disease (where advanced therapy has been shown to be more likely to cause harm), or to lung disease (where there is a lack of data showing benefit).

Before attempting advanced therapy, a specialized cardiac catheterization study is done to assess the "vasoreactivity" of the pulmonary circulation. During this test, drugs are given to assess whether the pulmonary blood vessels are capable of dilating. If so, certain types of advanced therapy are more likely to be effective.

Many drugs have been developed for treating pulmonary hypertension, including:

• Calcium channel blockers such as diltiazem and nifedipine
• Prostanoids such as Flolan (epoprostenol), Remodulin (treprostinil), and Ventavis (iloprost)
• Endothelin receptor antagonists such as Tracleer (bosentan), Letaris (ambrisentan), and Opsumit (macitentan)
• PDE5 inhibitors such as Viagra (sildenafil), Cialis (tadalafil), and Levitra (vardenafil)
• Guanylate cyclase stimulant such as Adempas (riociguat)

This long list of choices makes the process of selecting the "best" drug or the "best" combination of drugs exceedingly complex. Several factors have to be taken into consideration in making this selection, including the underlying disease, the severity of the pulmonary hypertension, the degree of vasoreactivity, whether certain drugs are covered by medical insurance, and the likely side effects. This is why such decisions should be made by doctors who are experts in treating pulmonary hypertension.

Finally, lung transplantation has been used successfully in some people with idiopathic pulmonary arterial hypertension. This is obviously a drastic and risky proposition, and is only attempted in patients who meet strict criteria designed to optimize the odds of survival. 

Summary

The optimal treatment for pulmonary hypertension can be difficult and complex. Patients with pulmonary hypertension do best who form a close working partnership with their doctors, taking an active role in deciding on therapy, in carefully observing and reporting on the status of their symptoms and functional capacity, and following the agreed upon treatment regimen very closely.