Pulmonary hypertension is a serious, often fatal disease. It occurs when the small arteries in the lungs develop changes in their walls: thickening of the muscle and of the innermost cell layer, leading to narrowing of the inside of the vessel. Eventually, the heart has to work so hard to get enough oxygen-rich blood to the lungs that patients often develop heart failure.

The first symptoms are usually shortness of breath (81%), fatigue (28%), chest pain or discomfort (20%), and swelling of the ankles and legs (20%). Most patients go to their doctor within a month of the first symptom. But, according to a report at the CHEST 2007 meeting of the American College of Chest Physicians, the average time until the possible diagnosis is made is 7 months.

When the diagnosis is seriously considered, right heart catheterization needs to be done for confirmation. This is usually first done, on average, 13 months after the first symptoms. Often specific treatment is delayed from 15 months to 3 years before the first symptom. Considering there are a number of drugs that are effective in pulmonary hypertension (including a close relative of Viagra®), this delay in diagnosis is unacceptable. One must assume that there are a number of reasons: lack of awareness/consideration of pulmonary hypertension by the doctor, reluctance to have right heart catheterization, and delay in transferring care to a clinic specialized in treating the disease. Pulmonary hypertension - either of unknown cause or secondary to another condition - is still relatively rare, but the outcome is often fatal. Let's hope the situation will improve as more physicians become aware of the problem...