FDA approval was based on data submitted from this study and another randomized, multi-center trial. Both studies were led by researchers at the University of Maryland Medical Center. Primary pulmonary hypertension is a disorder of the blood vessels inside the lungs. It makes it hard for blood to flow into the lungs, causing lung pressure to rise. Eventually, the condition can cause the right side of the heart to fail.
Patients experience shortness of breath, chest pain, and fainting.
PPH mainly affects young women in their 20's or 30's, often around or right after pregnancy. About 50 percent of people with primary pulmonary hypertension can be treated effectively with medications that dilate the vessels. The others may go on to become very ill and, until the approval of this new drug, their only other option was a lung transplant. While PPH is not a curable disease, it often can be treated quite effectively. This drug offers long term treatment for some patients or it can help stabilize other patients while they wait for a transplant.