The PAH community is fortunate to have many treatment options.  14 medications are approved and probably more coming.  But for some patients, medications are not enough.  We have long recognized that patients with congenital heart disease (abnormal communications in their heart) who have PAH have a much better prognosis. What is Atrial Septostomy? In certain parts of the … [Read more...]

Pulmonary arterial hypertension is caused by abnormal changes in the pulmonary arteries of patients. These changes include increased thickness of the muscle layer of the pulmonary arteries and increased stiffness of the pulmonary arteries.  Over the last 25 years, 14 medications have been approved to try and treat these changes. These medications have resulted in dramatic … [Read more...]

It has been some time since I last wrote about avoiding Stem Cell Therapy.  Recently several of my patients have been suckered into this trap. Stem cells are bone marrow derived cells that have the potential to divide and become cells of any part of our body.  There is great promise in trying to understand how to develop effective treatments that use these cells.  … [Read more...]

Pulmonary arterial hypertension at its core is a disease of abnormal lung blood vessel behavior. While our currently available therapies have successfully improved the quality of life of our patients and extended their life expectancy, we still have a long way to go.  PAH patients unfortunately still often face progressive symptoms over time. What is the C76 Molecule? The FDA … [Read more...]

After six years, the FREEDOM-EV clinical trial has finished and preliminary results have been released. This study included patients with PAH who were already on a single oral therapy (either a PDE 5 inhibitor or an ERA). They were then randomized to have either a placebo or Orenitram added to their regimen.  Although we don’t have all of the final results, United Therapeutics … [Read more...]