How pulmonary arterial hypertension develops and progresses

Development of induced pluripotent stem cell models to elucidate mechanisms and develop treatments for pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) is abnormally high blood pressure in the arteries carrying blood to the lungs, causing damage to the right side of the heart. Symptoms include breathlessness or tiredness, and can cause death from heart failure within a few years. It is hard to study this disease and search for new treatments, because it is difficult to collect blood vessels and heart cells from patients. If scientists do collect cells, they are from very poorly patients so doctors cannot work out how the disease has started. Dr Amer Rana and BHF Prof Nick Morrell from the University of Cambridge have been studying stem cells from both healthy people and PAH patients, that they can instruct to turn into the types of cells affected in PAH. They have now been awarded a grant to grow blood vessels from these stem cells in the lab, and look for differences between vessels made from healthy cells and diseased cells. They will also use these stem cells to develop a new tool to find and test new medicines for PAH. This research will reveal more about how this disease starts and progresses, and may result in a new drug screening tool that could ultimately reveal new ways to treat patients with this disease.