The rare lung disease called pulmonary hypertension occurs when the arteries responsible for transporting the blood from the heart to the lungs are narrowed and blocked. This means the blood cannot pass through the vessels properly, which results in a blood pressure increase of abnormally high levels. This process takes place in the pulmonary arteries and strains the right ventricle of the heart. The heart has two upper and two lower ventricles that pump the blood to the lungs and the rest of the body, which are affected by the disease.
Due to the disease, the right ventricle needs to work harder to pump the blood, becoming enlarged and gradually weakened. When the blood vessels become completely narrowed or blocked, the arteries become stiff and the heart loses the ability to properly pump blood to the lungs. This can cause right heart failure. To prevent and treat this life-threatening disease, it is important to determine the causes of pulmonary hypertension so that a proper diagnosis and prognosis can be determined by a specialist.
General Knowledge of Pulmonary Hypertension Causes
There is scientific evidence demonstrating that pulmonary hypertension may be related to injuries in the cell layer located in the small blood vessels of the lungs. But the explanation is not that simple. The reasons for the disease are not completely understood, but it is known that it alters the interaction between the cells in question and the muscle cells in the vessel wall, causing a contraction of the muscle. There are known causes that can contribute to the development of pulmonary hypertension.
Among these are the use of the appetite suppressant drug called “fen-phen” (dexfenfluramine and phentermine), which is banned in the United States. Chronic liver disease, liver cirrhosis and other liver diseases; scleroderma or systemic lupus erythematosus and other rheumatoid diseases; along with lung tumors, emphysema, chronic obstructive pulmonary disease (COPD), pulmonary fibrosis, or other lung conditions may also cause pulmonary hypertension.
Other diseases may contribute to the development of pulmonary hypertension, including aortic valve disease, left heart failure, mitral valve disease, congenital heart disease, as well as thromboembolic disease. Low-oxygen conditions, which can be caused by high altitude, obesity, sleep apnea or other factors, and genetics are also risk factors.
There are different types of pulmonary hypertension, and the reasons for the development of the disease are not always clear.
Different Types of Pulmonary Hypertension Causes
- Idiopathic Pulmonary Hypertension is when there is a lack of explanation for the disease. Idiopathic means the reason for the disease cannot be found. However, it is known that some of patients carry a gene that comprises a risk factor for pulmonary hypertension, and in the group that classifies idiopathic pulmonary hypertension is the subtype of disease known as familial pulmonary hypertension.
- Secondary or Associated Pulmonary Hypertension is a disease type caused by a primary medical problem. It is more common than idiopathic pulmonary hypertension. The most usual primary diseases that can result in secondary pulmonary hypertension include blood clots in the lungs, also known as pulmonary emboli; chronic obstructive pulmonary diseases (COPD) like emphysema; scleroderma; lupus or other connective tissue disorders; sleep disorders; congenital heart defects; sickle cell anemia; chronic liver disease, also known as cirrhosis; AIDS; lung diseases like pulmonary fibrosis; and left-side heart failure. The use of stimulant drugs like cocaine can also cause this subtype of pulmonary hypertension.
- Eisenmenger Syndrome and Pulmonary Hypertension is a congenital medical condition. When patients are born with this heart defect, there is a higher risk of developing a large hole between the two lower heart ventricles, which is called a ventricular septal defect (VSD). As a result, the blood circulation in the heart is affected, and the oxygen-poor blood (blue blood) from the right side of the heart flows into the left ventricle and is pumped to your body so you don’t receive enough oxygen to all your organs and tissues. While normally the oxygen-rich and oxygen-poor types of blood circulate separately and the blood is supposed to be transported throughout the entire body, it keeps returning to the lungs, increasing pressure in the pulmonary arteries and causing pulmonary hypertension.
Note: Pulmonary Hypertension News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.
- PHA Guide Aims to Help Newly Diagnosed Patients, Their Caregivers and Family
- VivaLNK’s Wireless Heart Monitor Can Now Be Used for Six-minute Walk Test
- Rare Disease Day ‘Patient Hero’ Raises Awareness Through Fashion
- Insmed Announces Top-line Results from Phase 1 Trial of TPIP
- Rare Disease Day at NIH, Set for March 1, Growing Year by Year
- FDA Approves Alembic’s Generic Injection of Remodulin for PAH
- Remunity Pump on Market for Dispensing Remodulin
Find Out More