Primary pulmonary hypertension, or PPH, is a progressive disorder of the arteries that feed blood to the lungs. It is most common in women between the ages of 20 and 40, and 500 to 1,000 new cases are diagnosed every year in the United States. Even though it mostly affects women, it can occur in men and women of any age, including young children with no other known risk factors. PPH is a vaguely understood disease and is difficult to diagnose. A doctor who does not conduct a sufficiently thorough examination will likely miss the disorder, causing a proper diagnosis to be delayed by several years.

PPH is also known as idiopathic pulmonary arterial hypertension, or IPAH. Doctors only have a basic knowledge of this form of primary pulmonary hypertension, as it often appears without any other underlying heart or lung condition. The World Health Organization has stated that appetite suppressing drugs like fenfluramine, dexfenfluramine and aminorex are risk factors for the condition. In fact, some experts believe that taking these drugs for only three months can increase the risk of PPH by 30 times. However, some doctors now believe that many people suffering from IPAH have inherited a genetic defect from one or both parents. People who have suffered injury to an artery may also be at a greater risk of developing the disease.

At first, PPH is not life-threatening. Like many other forms of cardiac disease, it takes several years for severe complications to become noticeable. Primary pulmonary hypertension is marked by increased blood pressure in arteries connecting the lungs and heart. This increase in blood pressure is due to vasoconstriction of blood vessels and, eventually, fibrosis of the vessels. The heart, as a result, has to work harder to deliver enough oxygen to the body. This further increases blood pressure and causes an enlarged heart.

At this point, cardiac failure is a real danger, especially during physical activity. Eventually, the heart is not able to keep up with the oxygen demands of the lungs, as vasoconstriction, fibrosis and heart hypertrophy have all reduced the circulatory system’s ability to feed oxygen to lung tissue. This eventually results in right-sided heart failure.

The symptoms of primary pulmonary hypertension appear relatively minor in the beginning. The most common early signs are fatigue, elevated heartbeat and chest pain. Because these symptoms are slow to develop and can be masked by other conditions, doctors often misdiagnose patients. Over time, these symptoms will worsen, and the patient may also experience lightheadedness following routine physical activity, fainting and swelling in the legs and ankles. He or she may also show blue lips or skin, suggesting oxygen deprivation to parts of the body.

There is no cure for primary pulmonary hypertension, and the disease can be fatal if it is not caught early. Some patients may require a heart or lung transplant to prevent organ failure. To catch this disease before it becomes fatal, a doctor needs to provide a full battery of tests and must have a thorough medical history of the patient. Unfortunately, some doctors neglect to do this, putting their patients at risk. Anyone who has been diagnosed with PPH may want to seek out a legal professional to determine what options he or she has regarding restitution.