What Are the Treatments for PPH?

Pulmonary arteries carry blood from the heart to the lungs. When blood pressure rises extremely high in a pulmonary artery for no apparent reason, the condition is known as PPH, or primary pulmonary hypertension. It is a rare condition; in the United States only about 500 to 1,000 new cases are discovered each year. Its exact cause has not been determined, in part due to how infrequently it occurs.

Description

• Abnormally high pulmonary blood pressure, or hypertension, is associated with changes in tiny capillaries in the lungs. Healthy capillaries have an inner lining that keeps the blood from leaking out. The muscle cells on the outer part of the capillaries allow them to expand and contract as blood passes through. In PPH, the inner lining starts coming off, causing the blood to escape into the muscles. The muscles then contract, elevating blood pressure.

Symptoms

• The symptoms of PPH surface very slowly, making the disease difficult to treat. Early on, a victim may experience fatigue or weariness, followed by breathing problems. Dizziness is a symptom of PPH but may also be indicative of some other ailment. Symptoms are swelling in the lower extremities and bluish discoloration of the skin. These symptoms may be related to some other type of malady--heart disease, for example. PPH is categorized as a lung disease even though it involves the heart.

Evaluation and Treatment

• Treating patients with PPH depends largely upon what is discovered in the initial evaluation, which should be done at a medical facility having expertise in this area. Pulmonary arterial pressure is evaluated before treatment can begin.

  Treatments focus on the dilating--or relaxation--of blood vessels in the lungs, and they vary depending on the individual. Initially, doctors try various modalities (tendencies to conform to a general pattern or belong to a particular group or category) in order to tailor therapy to each patient. Then, as treatment progresses, doctors may make changes as the patient's condition changes.

  A class of medication known as calcium channel blockers lowers blood pressure by preventing calcium from getting into the cells. This has the effect of relaxing and widening capillaries so that blood can more easily pass through them. If calcium channel blockers are not working well, intravenous delivery of prostacyclin--a vasodilator--may help dilate capillaries. Doctors use a portable device for continuous delivery of this substance. They also may use delivery of prostacyclin as an interim method before initiating a transplant--either lung, or heart and lung. These are last-resort measures to handle PPH.

  Anticoagulants and diuretics may help treat this disease--anticoagulants because their anti-clotting properties promote blood flow, and diuretics because they reduce fluid's burden on the heart. If the patient is having breathing problems, administering oxygen has definite benefits. Some patients need it 24 hours a day.