Primary Pulmonary Hypertension

Some patients do well by taking medicines that make the work of the right ventricle easier. Anticoagulants, for example, can decrease the tendency of the blood to clot, thereby permitting blood to flow more freely. Diuretics decrease the amount of fluid in the body, further reducing the amount of work the heart has to do.

Until recently, nothing more could be done for people who have PPH. However, today doctors can choose from a variety of drugs that help lower blood pressure in the lungs and improve the performance of the heart in many patients.

Some patients also require supplemental oxygen delivered through nasal prongs or a mask if breathing becomes difficult; some need oxygen around the clock. In severely

affected cases, a heart-lung, single lung, or double lung transplantation may be appropriate.

• Drugs: Doctors now know that PPH patients respond differently to the different medicines 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 may also have to be changed.

To find out which medicine works best for a particular patient, doctors evaluate the drugs during cardiac catheterization. This way they can see the effect of the medicine on the patient's heart and lungs. They can also adjust the dose to reduce the side effects that may occur — for example, systemic low blood pressure (hypotension); nausea; angina; headaches; or flushing.

To determine whether a drug is improving a patient's condition, both the pulmonary pressure and the amount of blood being pumped by the heart (the cardiac output) must be evaluated. A decrease in pulmonary pressure alone, for example, does not necessarily mean that the patient is recovering; cardiac output must either increase or remain unchanged. The most desirable response is a decrease in pressure and an increase in cardiac output. Once the patient has reached a stable condition, he or she can go home, returning every few weeks or months to the doctor for followup.

At present, approximately one-quarter to one-half of patients can be treated with calcium channel-blocking drugs given by mouth. By relaxing the smooth muscle in the walls

of the heart and blood vessels, these calcium channel blockers improve the ability of the heart to pump blood.

A vasodilator, prostacyclin, is helping some severely ill patients who are unresponsive to treatment with calcium channel blockers. The drug, which has been studied in clinical trials, imitates the natural prostacyclin that the body produces on its own to dilate blood vessels. Prostacyclin also seems to help prevent blood clots from forming.

Prostacyclin is administered intravenously by a portable, battery-operated pump. The pump is worn attached to a belt around the waist or carried in a small shoulder pack. The medication is then slowly and continuously pumped into the body through a permanent catheter placed in a vein in the neck.

Protstacyclin seems to improve pulmonary hypertension and permit more physical activity. It is sometimes used as a bridge to help those patients waiting for a transplant, while in other cases it is used for long-term treatment.

• Transplantation: The first heart-lung transplant was performed in this country in 1981. Many of these operations were performed for patients with PPH. The survival rate is the same as for other patients with heart-lung transplants, about 60 percent for one year, and 37 percent for five years.

The single lung transplant is the most common method of transplant used in cases of PPH. This procedure, in which one lung is replaced, was first performed in 1983 in patients with pulmonary fibrosis. Double lung transplants are also done to treat PPH.

There are fewer complications with the single lung transplant than with the heart-lung transplant, and the survival rate is on the order of 70 percent for one year. A surprising finding is the remarkable ability of the right ventricle to heal itself. In patients with lung

transplants, both the structure and function of the right ventricle markedly improve. Complications of transplantation include rejection by the body of the transplanted organ and infection. Patients take medications for life to reduce their body's immune system's ability to reject "foreign" organs.

With the cause of primary pulmonary hypertension still unknown, there is at present no known way to prevent or cure this disease. However, many patients report that by changing some parts of their lifestyle, they can go about many of their daily tasks. For example, they do relaxation exercises, try to reduce stress, and adopt a positive mental attitude.

People with PPH go to school, work at home or outside the home part-time or full-time, and raise their children. Indeed, many patients with PPH do not look sick, and some

feel perfectly well much of the time as long as they do not strain themselves physically.

Walking is good exercise for many patients. Some patients with advanced PPH carry portable oxygen when they go out; patients who find walking too exhausting may use a wheelchair or motorized scooter. Others stay busy with activities that are not of a physical nature.

For the patient who lives at a high altitude, a move to a lower altitude — where the air is not so thin, and thus the amount of oxygen is higher — can be helpful. Medical care is important, preferably by a doctor who is a pulmonary vascular specialist. These specialists are usually located at major research centers.

PPH patients can also help themselves by following the same sensible health measures that everyone should observe. These include eating a healthy diet, not smoking, and getting plenty of rest. Pregnancy is not advised because it puts an extra load on the heart. Oral contraceptives are not recommended, and other methods of birth control should be used.

Most doctors and patients agree that it is important for both patient and family to be as informed as possible about PPH. In this way everyone can understand the illness and apply that information to what is happening. In addition to family and close friends, support groups can help the PPH patient.

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