Primary Pulmonary Hypertension
(Say THAT three times fast!)
If you don't know much about primary (i.e. unexplained) pulmonary hypertension, lucky you!
Primary pulmonary hypertension (PPH) is a rare lung disorder in which the blood pressure in the pulmonary artery rises far above normal levels for no apparent reason. For example, the normal average is about 25mmHg. Prior to my transplant, my pulmonary pressure was 130-140mmHg. That is 7-8 times the normal pressure!!! Sometimes my heart would beat so hard you could see it through my chest!
The scary part -- well, one scary part, anyway -- is that we don't know the cause. There may be one or more causes. But the symptoms give it away. The first symptom is often fatigue, difficulty breathing, dizziness and even fainting spells. Then they progress to swelling in your legs, blue lips and skin, and chest pain. I know that sounds a little like one of those pharmeceutical commercials, but it's true. And it's not much fun. Even with the blaring symptoms, PPH is commonly misdiagnosed as bronchitis, depression, or just aging.
Below is a picture of my lungs on the left, compared to a normal lung on the right. Notice the very prominent and enlarged blood vessels (white) in my diseased lung.
The next photo is of me comparing my lungs to a normal set of lungs. The pathologist helped me to understand how the hardened vessels in my lungs caused the pressure in my lungs to build up making it much more difficult for my heart to pump blood into my lungs through the pulmonary artery. This is what led to right side of my heart becoming enlarged.
There's a lot more to know about PPH. So if you're really interested, check out a few of these sites: