Doctors can choose from a variety of drugs that help lower blood pressure in the lungs and improve heart performance in many patients.
Physicians now know that patients with PPH respond differently to the different medications that dilate or relax blood vessels and that no one drug is consistently effective in all patients.
Because individual reactions vary, different drugs have to be tried before chronic or long-term treatment begins.
During the course of the disease, the amount and type of medicine also may have to be changed.
To find out which medicine works best for a particular patient, doctors evaluate the drugs during cardiac catheterization.
- At present, about one-quarter of patients can be treated with calcium channel-blocking drugs given orally.
- Intravenous prostacyclin is a vasodilator. It helps patients who don't respond to treatment with calcium channel blockers given by mouth. It's continuously delivered by a portable, battery-operated infusion pump. Despite this complexity, prostacyclin improves pulmonary hypertension and permits increased physical activity. This improves the quality of life for patients of all ages. Prostacyclin is sometimes used as a bridge to help patients waiting for a transplant. In other cases it's used for long-term treatment.
Besides oral calcium channel blockers and chronic intravenous prostacyclin, clinical trials are under way to evaluate new drugs to improve the treatment of PPH.
Some patients also do well by taking medicines that make the right ventricle's work easier.
- Anticoagulants, for example, can decrease the tendency of the blood to clot, thus permitting the blood to flow more freely.
- Diuretics reduce the amount of fluid in the body, further reducing the amount of work the heart has to do.
- Some patients also require supplemental oxygen delivered through nasal prongs or a mask if breathing becomes difficult. Some need oxygen around the clock.
Transplantation (heart-lung or lung) is reserved for patients who don't respond to medical therapy. The decision whether a patient requires heart-lung transplantation or lung transplantation is made after a thorough evaluation at a lung transplantation center.
What are the long-term prospects for patients with primary pulmonary hypertension?
Despite the complexity of some of the various medical therapies, accurate, early diagnosis and initiation of treatment have saved the lives of many patients with PPH.
With optimal medical and/or surgical therapy, patients can often return to a virtually normal lifestyle, including running a household, returning to school and participating in many physical activities.
Most doctors and patients agree that it's very important for both patients and families to be as informed as possible.
This lets everyone understand the illness and apply the information to what is happening. In addition to family and close friends, support groups can help PPH patients.
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