What is Pulmonary Hypertension?

I thought I would explain what is pulmonary hypertension. When pulmonary hypertension is diagnosed with no known cause, it is called primary pulmonary hypertension. When the cause is known, such as with me, the cause is Scleroderma, then it is known as secondary pulmonary hypertension. It is rare and without treatment it is usually a rapid fatal disease.
Blood enters the right side of your heart carrying very little oxygen. The right side of your heart pumps the blood into the arteries of your lungs. (Here is a fact: this is the only place in your body where your blood is blue). These arteries grow smaller and become arterioles, about the size of a hair. They are so small, the red blood cells can only go through one or two at a time. They then go through even tinier capillaries that surround the air sacs in your lungs. Here they take on oxygen from the lungs, return to the left side of the heart and are pumped out to the rest of your body.
PH usually starts in the lungs. My lungs are scarred from the Scleroderma and they don’t have the elasticity your lungs have nor the ability to move the oxygenated blood through the cells due to narrowing and scarring (fibrosis). My arteries and arterioles are stiff and scarred as well restricting blood flow even more. So I had high blood pressure in my lungs first as my lungs worked harder. When the lungs can’t work well, the pressure backs up into the heart which works harder to compensate for the lungs to push blood through. Eventually, your heart can’t keep up and there is less blood circulating in the lungs to pick up oxygen and you become tired, dizzy, and short of breath. Fainting, edema, chest pain, and dry cough may be present. You may feel like you are choking or hungry for air. I can feel my heart beat, clear into my eyeballs. It will thump, flutter and do other strange things. I have had fainting problems for several years. I get up to do something and I can feel the blackness closing in. I have to lay down for a while then get up and try again. It usually goes away. My oxygen tank has helped a lot.
Not much is known about PH, even less is known about PH with Scleroderma. It is a disease of the blood vessels of the lungs and heart. Tomorrow I will talk about the little bit that is known about Scleroderma and PH.