Primary pulmonary hypertension (PPH) is a rare lung disorder in which the blood pressure in the pulmonary arteries rises above normal levels, creating additional stress and work for the heart. It occurs in the absence of other diseases in the heart and lungs. It is marked by shortness of breath, dizziness, and fainting, all worsened by exertion or exercise. According to the American Lung Association, there were 3,065 deaths attributable to PPH in 2000. PPH usually affects women between the ages of 21 and 40.
No one knows exactly what causes PPH. It is linked to familial history, immune system diseases, and the ingestion of certain drugs. Appetite suppressant use, especially a dosage lasting more than three months, is linked to increase in the incidence of PPH and heart valve damage.
The prognosis for people diagnosed with PPH varies. The median survival period is three years after diagnosis; however, the survival rate is longer for those without heart failure and for those diagnosed after the age of forty.
Treatment for primary pulmonary hypertension cannot cure or halt the progression of the disease but can relieve the symptoms. Some drugs know as vasodilators dilate the blood vessels in the lungs. This dilation helps to reduce blood pressure, helping to relieve strain on the heart as well. However, such drugs must be evaluated with heart tests, as they can cause serious side effects in some patients. Another shortcoming of vasodilators is that they rarely normalize the elevated blood pressure and must be consumed in high doses to achieve the desired effect.
A blood thinning drug is also another treatment option for those diagnosed with PPH. Anticoagulants, or drugs than discourage blood clotting, especially in the lungs, may be beneficial. Supplemental oxygen is often recommended by doctors as low blood oxygen levels is a side effect of PPH. Lung transplants are a viable option for patients with severe PPH.
Sometimes, if the patient's heart is also diseased, a heart-lung transplant may also be performed. Lung transplant is an extreme measure for patients with life-threatening PPH. To qualify for a transplant, patients aged twelve and older are evaluated according to the Lung Allocation Scoring (LAS) system which evaluates their health and need. Lungs are then awarded based on patient need, rather than how long they have been on a waiting list. Survival rates are generally very high at the one year mark but lower at five and ten year follow-ups. Transplanted lungs usually last three to five years before showing signs of failure.
