<International Circulation>: Because primary pulmonary hypertension is unexplained by name and nature， treatment is limited and difficult. What are the latest methods of treating pulmonary hypertension?

    Prof Wilkins:There has been a paradigm shift in our approach to idiopathic pulmonary arterial hypertension. The treatments that are currently available are focused on research conducted over the last ten or twenty years addressing vascular motor tone. We have attempted to tackle the pulmonary vasoconstriction we know is associated with pulmonary arterial hypertension. However， histological analysis of patients who have died with the disease has revealed marked remodeling of the blood vessels， so now we are beginning to focus more and more on how to directly tackle the vascular proliferation that drives that remodeling and structural changes in the lung. We are borrowing ideas from oncology and other disciplines in order to come to grips with this. Some of the most exciting things that are happening at the moment centre on tyrosine kinase inhibitors， which are in early phase clinical trials in man. The early data in animals is suggesting much promise. I think that promise will be balanced by reality as these studies progress but none-the-less， we appear to have a new therapeutic prospect for this disease. In a broader sense， there is a lot of interest in what we might do with stem cells and progenitor cells in the setting of regenerative medicine. While we focus very strongly on the remodeling of pulmonary vessels， we shouldn’t forget the heart and in particular the right ventricle. It is the preservation of right ventricular function which is the key to survival in this disease. We may be able to do this with drugs， but we may be able to do it more effectively with cell replacement therapy. At this stage， very experimental and a lot of hype associated with it， but a promising therapeutic approach.

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