Pulmonary Arterial Hypertension
Pulmonary hypertension is high blood pressure that only occurs in the lungs blood vessels. It affects the arteries in your lungs and puts tremendous pressure on the right side of your heart which ultimately fails.
In one form of pulmonary hypertension, tiny arteries in your lungs, called pulmonary arterioles, and capillaries become narrowed, blocked or destroyed. This makes it harder for blood to flow through your lungs, and raises pressure within your lungs’ arteries. As the pressure builds, your heart’s lower right chamber (right ventricle) must work harder to pump blood through your lungs, eventually causing your heart muscle to weaken and fail.
Some forms of pulmonary hypertension are serious conditions that become progressively worse and are sometimes fatal. Although some forms of pulmonary hypertension aren’t curable, treatment can help lessen symptoms and improve your quality of life.
Risk Factors
✓ Idiopathic pulmonary arterial hypertension is more common in younger adults
✓ You have one of various conditions that can increase your risk of developing pulmonary hypertension
✓ You use illegal drugs, such as cocaine
✓ You take certain appetite-suppressant medications
✓ You have a family history of the disease
Causes
Your heart has two upper chambers (atria) and two lower chambers (ventricles). Each time blood passes through your heart, the lower right chamber (right ventricle) pumps blood to your lungs through a large blood vessel (pulmonary artery).
Chambers of the heart
In your lungs, the blood releases carbon dioxide and picks up oxygen. The oxygen-rich blood then flows through blood vessels in your lungs (pulmonary arteries, capillaries and veins) to the left side of your heart. Ordinarily, the blood flows easily through the vessels in your lungs, so blood pressure is usually much lower in your lungs.
With pulmonary hypertension, the rise in blood pressure is caused by changes in the cells that line your pulmonary arteries. These changes can cause the walls of the arteries to become stiff and thick, and extra tissue may form. The blood vessels may also become inflamed and tight.
These changes in the pulmonary arteries can reduce or block blood flow through the blood vessels. This makes it harder for blood to flow, raising the blood pressure in the pulmonary arteries.
Insight
Pulmonary hypertension, also known as pulmonary arterial hypertension, is a rare condition affecting 1 to 2 people in every 1 million over the US and Europe. Older women are at higher risk of the condition, but it can affect men and women of all ages.
It is characterized by high blood pressure in the pulmonary arteries, which carry oxygen and blood from the heart to the lungs. This means the right side of the heart has to work harder to pump blood around the body.
The condition typically occurs alongside other diseases, such as heart and blood vessel diseases, lung diseases and liver diseases. Pregnancy, sleep apnea, birth defects of the heart and certain autoimmune disorders, such as rheumatoid arthritis, are also common causes of pulmonary hypertension.
Symptoms of the condition include tiredness, chest pain, shortness of breath during routine activity, reduced appetite, a fast heartbeat and pain on the upper right side of the abdomen.
ALT-100 Benefits
Lung inflammation is now recognized as a key contributor to the pulmonary vascular remodeling that is essential to the development of Pulmonary Arterial Hypertension and contributing directly to Pulmonary Arterial Hypertensions’s unacceptably high mortality. Aqualung Therapeutics Corporation scientists, via genomic–intensive approaches, identified nicotinamide phosphoribosyltransferase (Nampt) as a novel candidate gene and cytokine in Pulmonary Arterial Hypertension. Pulmonary Arterial Hypertension subjects exhibit increased blood Nampt protein levels and Nampt is robustly expressed in lung endothelial cells (EC) of remodeled vessels from Pulmonary Arterial Hypertension subjects. Aqualung Therapeutics will utilize the human monoclonal antibody ALT-100 as a targeted therapy that focuses on inflammatory pulmonary vascular remodeling, a key feature of the Pulmonary Arterial Hypertension pathobiology. Neutralizing Nampt in Pulmonary Arterial Hypertension will attenuate lung inflammation and vascular remodeling via reduced signaling through its receptor, Toll like receptor 4 (TLR4). Nampt dysregulates processes implicated in pulmonary vascular remodeling such as resistance to apoptosis, cell proliferation, and smooth muscle cell survival.