Once the underlying cause is identified, your doctor will need to assess how severe your pulmonary hypertension is. This is usually done by performing an echocardiogram, which will provide an estimate of your pulmonary artery pressure, and an exercise test to measure your baseline functional capacity. This baseline assessment will help your doctors to decide how aggressive to be with treatment, and to measure how well you respond to it.
Treatment Aimed at the Underlying Medical ConditionIt is likely that aggressively treating the underlying cause of your pulmonary hypertension will be the most critical part of your treatment. It is important for you to learn as much as you can about the medical disorder that is producing your pulmonary hypertension, so you can partner with your doctors in deciding on the best possible therapy. Following the links provided in this discussion of the causes of pulmonary hypertension will get you started.
Treatments Often Useful for Anyone With Pulmonary HypertensionIn addition to therapy aimed specifically at treating the underlying cause, there are treatments that are used more generally in many patients with 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 shortness of breath 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 hypoxemia (reduced blood oxygen levels). When hypoxemia 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 hypoxemia, 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.
- 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. Doctors may overlook this very useful aspect of therapy, so be sure to bring it up to your doctor and ask for specific instruction on exercise that is safe for you.
Advanced Therapy"Advanced therapy" for pulmonary hypertension is aimed at the pulmonary hypertension itself, rather than the underlying cause. Advanced therapy is called "advanced" not because it is more effective than the more general therapies, but because it is complex, relatively risky, very expensive, and often inconvenient (for instance, it may require intravenous therapy). Advanced therapy should only be administered by physicians who are expert in treating pulmonary hypertension.
Your doctor may consider advanced therapy if you have 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, if you have primary pulmonary hypertension).
Advanced therapy will probably not be considered at all if you have pulmonary hypertension 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).
If your doctor has recommended advanced therapy, you will need to have a specialized cardiac catheterization study aimed at assessing the "vasoreactivity" of your pulmonary circulation. That is, during the test you will be given drugs, such as adenosine, to assess whether your pulmonary blood vessels are capable of dilating. If your pulmonary blood vessels can be dilated (that is, they are "vasoreactive"), 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 epoprostenol (Flolan), treprostinil (Remodulin) and iloprost (Ventavis)
- Endothelin receptor antagonists such as bosentan (Tracleer) and ambrisentan (Letaris)
- PDE5 inhibitors such as sildenafil (Viagra), tadalafil (Cialis), and vardenafil (Levitra)
SummaryObviously, the optimal treatment for pulmonary hypertension can be difficult and complex. Patients with pulmonary hypertension do best who form a working partnership with their doctors. It is important that you take as active a role as possible in deciding on therapy, carefully observing and reporting the status of your own symptoms and functional capacity, and following your treatment regimen very closely.
Galie, N, Hoeper, MM, Humbert, M, et al. Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J 2009; 34:1219.