Pulmonary hypertension (PH) or pulmonary arterial hypertension (PAH) is a serious, progressive disease in which there is elevated blood pressure in the arteries supplying to the lungs. The blood flow from the heart to the lungs is disrupted due to constricted blood vessels. This causes extra load on heart for blood pumping to the lungs, eventually leading to weakness of heart muscles. This leads to difficulty exchanging oxygenated blood from deoxygenated blood leading to less supply of oxygenated blood to different tissue of the body. This leads to symptoms of fatigue, dizziness and shortness of breath in patients suffering from pulmonary hypertension.
Types and Causes of Pulmonary Hypertension
There are five types of pulmonary hypertension ranging from type 1 to type 5. Type 1 relates to numerous causes including connective tissue diseases (autoimmune diseases), liver diseases, congenital heart diseases, sickle cell anemia, HIV infection, schistosomiasis (parasitic infection), due to drugs and certain toxins (include some of diet pills and recreational drugs) and diseases affecting small blood vessels of the lungs. PH can also be idiopathic (with no known cause) and can have a genetic propensity.
Type 2, PH is caused by conditions affecting left side of the heart including mitral valve disease and chronic systemic elevated blood pressure.
Type 3, PH is related to various breathing and lung conditions such as chronic obstructive pulmonary disease (COPD), pulmonary fibrosis (interstitial lung diseases) and sleep apnea.
Type 4, pulmonary hypertension is associated with clotting disorders such as blood clots in the lungs.
Type 5, pulmonary hypertension includes blood disorders (polycythemia vera and thrombocythemia), metabolic disorders including thyroid diseases and glycogen storage disease, systemic disorders (such as sarcoidosis and vasculitis), kidney diseases and other conditions including tumors causing compression of pulmonary arteries.
In addition, when PH is triggered by pre-existing disease, it is known as secondary pulmonary hypertension.
How long Do You Live with Pulmonary Hypertension?
Unfortunately, even with advancement in medical field there is no permanent treatment for PH. Medications, lifestyle changes and proper management can only limit/slow the progression of disease. The prognosis of the disease is very poor, if it is left untreated and the patient suffering from it dies within 3 years of diagnosis. If the patient is in low risk group then the patient can live for over 10 years. The survival rate of the patient with pulmonary hypertension depends on the etiology. There is no definitive way to predict the survival rate of a patient suffering from PH. Data recovered from a large group of patients in early 2000s reveal, overall, one year survival rate as 85%, three year survival rate as 68%, five year survival rate as 57% and seven year survival rate as 49%.
The data also revealed that the prognosis is poor for patients whose etiology includes family history, connective tissue disorder or related to liver disease as compared to congenital heart disease. Poor prognosis is seen in patients with pericardial effusion as compared to patients who do not have pericardial effusion. The prognosis is poor in patients whose exercise capacity and distance covered in a 6-minute walk is less as compared to patients who have greater six-minute walk distance.
The prognosis is also worse for patients who have very high right atrial pressure and pulmonary vascular resistance as compared to patients who have low values of them. Patients with impaired kidney function and elevated levels of BNP (Brain Natriuretic Peptide) have a very poor prognosis too as compared to patients who have normal kidney function or who have low BNP.
However, the survival rates of patients with pulmonary hypertension has drastically improved with the advent of medications and improved quality of life. It was not until 1994, the first medication (Flolan) was introduced for the management of pulmonary hypertension and in the past two decades, there have been over ten medications available for the treatment of it. Hopefully, with more research and studies the survival rate of patients with PH will continue to improve.
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