Pulmonary hypertension, or PH, is a simplified name for a complex health problem: high blood pressure in the lungs. The name comes from pulmonary, or having to do with the lungs, and hypertension, which means high blood pressure. It is a disease that affects people of all ages and ethnic backgrounds.
PH starts when the small vessels that supply blood to the lungs constrict, or tighten up. It is more difficult for blood to get through to the lungs, and the heart must pump harder to overcome the resistance. As time passes, scarring (or fibrosis) of the vessels makes them stiffer and thicker, and some may be completely blocked. The extra stress causes the heart to enlarge and become less flexible. Less and less blood is able to flow out of the heart, through the lungs, and into the body, and more and more symptoms start to show.
In 1988, the World Health Organization (WHO) sponsored a meeting of PH specialists with the goal of creating a standardized system for defining different kinds of PH. The committee proposed that the disease be divided into the following five categories of pulmonary hypertension:
1. Pulmonary arterial hypertension (PAH)
2. Pulmonary hypertension associated with disorders of the respiratory system
3. Pulmonary hypertension due to chronic thrombotic or embolic disease
4. Pulmonary hypertension due to disorders directly affecting the pulmonary blood vessels
5. Pulmonary venous hypertension (PVH)
PH is often not diagnosed in a timely manner because its early symptoms can be confused with those of many other conditions. Some of the symptoms of PH include:
To establish a diagnosis of PH, a series of tests are performed that show how well a person's heart and lungs are working. These tests may include
If these tests show that a person definitely has PH, other kinds of diagnosis tests may be ordered to aid in identifying the cause, including:
Many Pulmonary Hypertension patients are diagnosed with PH in association with one or more related conditions. Research shows that up to 30% of Schleroderma patients, 20-40% of sickle cell patients and one out of every 200 HIV patients develop at least mild PH.
Other conditions commonly associated with PH included mixed connective tissue disease, congenital heart disease (CHD) chronic obstructive pulmonary disease (COPD), hereditary hemorrhagic telangiectasia (HHT), sleep apnea, liver disease and lupus. PH has also been linked to the use of certain drugs and toxins, including Fenfluramine, Dexfenfluramine and methamphetamines.