Pulmonary hypertension is rare blood vessel disorder

[bluemoon24]

Pulmonary hypertension (PH) is a rare blood vessel disorder of the lung in which the pressure in the pulmonary artery (the blood vessel that leads from the heart to the lungs) rises above normal levels and may become life threatening.



PH starts when the small vessels that supply blood to the lungs constrict, or tighten up. It is more difficult for the blood to get through to the lungs, and the heart must pump harder to overcome the resistance. As time passes, scarring (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 begin to show.



Symptoms of pulmonary hypertension include shortness of breath with minimal exertion and sometimes at rest, fatigue, chest pain, dizzy spells and fainting. Swollen ankles and legs and chest pain, especially during physical activity, are also symptoms of|pulmonary|hyperten-

sion.



Early symptoms may be confused with many other conditions, and pulmonary hypertension is frequently misdiagnosed. This can lead to further progression of the disease to a late stage by the time it is accurately diagnosed. Most types of PH are often not recognized until they are quite advanced. As a result, many people may have PH without knowing it, so higher awareness and earlier diagnosis can help people get earlier treatment.



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: Echocardiogram; electrocardiogram (ECG), right heart catheterization, a six-minute walk test, and assessment of function in tasks of daily living. Other tests may be ordered to aid in identifying the cause of PH, including: A computed tomography (CT or CAT) scan to rule out a pulmonary embolism or lung disease; a pulmonary function test to rule out obstructive lung disease; a formal sleep study to rule out sleep apnea; and laboratory tests to rule out hepatitis, collagen disease, HIV, or other conditions.



When no cause is found, the diagnosis is primary pulmonary hypertension (PPH).



The disease can be inherited as well. Secondary pulmonary hypertension (SPH) means the cause is known. A common cause of SPH are the breathing disorders emphysema and bronchitis. Other less frequent causes are the inflammatory or collagen vascular diseases such as scleroderma, Crest syndrome and systemic lupus erythematosus (SLE). Congenital heart diseases, chronic pulmonary thromboem bolism (old blood clots in the pulmonary artery), HIV infection, liver disease and diet drugs like fenfluramine and dexfenfluramine are also causes of pulmonary hypertension. Use of cocaine, methamphetamine or other street drugs can lead to pulmonary hypertension.



PH is a chronic condition that can become life-threatening. Although there is no cure, there are several treatments available, and patients have more options than ever before. There is a new medication in pill form, called Bosentan, that has been developed to decrease the pressure in the lungs. For select patients, this may be a treatment option. Most treatments for PH are prescribed for specific problems caused by the disease. If there is a combination of symptoms, then different types of medications may be prescribed, such as:



Anticoagulants to prevent blood clots to the lungs; calcium channel blockers to relieve constriction in pulmonary arteries; diuretics to reduce the fluid in the system; inhaled oxygen to make more oxygen available to the blood.



A number of drugs have been developed specifically to address symptoms of PH. For patients who do not respond to any of these treatments, a lung transplant may be recommended. The length of survival is continuing to improve, with some patients able to manage with the disorder for 15 to 20 years or longer.



Kaynak: SağlıkHaber