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    WSPF Pulmonary Hypertensive Vascular Disease: Pathobiology

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    автор | дата 23.02.2016
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    Please visit: www.openpediatrics.org OPENPediatrics™ is an interactive digital learning platform for healthcare clinicians sponsored by Boston Children's Hospital and in collaboration with the World Federation of Pediatric Intensive and Critical Care Societies. It is designed to promote the exchange of knowledge between healthcare providers around the world caring for critically ill children in all resource settings. The content includes internationally recognized experts teaching the full range of topics on the care of critically ill children. All content is peer-reviewed and open access-and thus at no expense to the user. For further information on how to enroll, please email: openpediatrics@childrens.harvard.edu Please note: OPENPediatrics does not support nor control any related videos in the sidebar, these are placed by Youtube. We apologize for any inconvenience this may cause. Welcome to World Shared Practices Forum. I'm Dr. Jeff Burns, Chief of Critical Care at Boston Children's Hospital and Harvard Medical School. We're very pleased to have with us today Dr. Jeff Fineman. Dr. Fineman is Professor of Pediatrics at the University of California in San Francisco and Chief of Pediatric Critical Care at the Benioff Children's Hospital in San Francisco. Jeff, welcome. Thank you very much. It's an honor to be here and be part of such a tremendous forum that you have here. Jeff, you are widely considered to be one of the most focused researchers in the field of pulmonary hypertension in our field of pediatric critical care. You've lectured all over the country and indeed all over the world on this. And many of my colleagues are undoubtedly wondering, how can we think about pulmonary hypertension? What are the latest thoughts about the state of the research and how we approach patients at the bedside? And so I'll begin simply by saying where do we begin? How do you think about pulmonary hypertension? Well, Dr. Burns, as you know, this is a, it's a complex disease. It can be a disease that's the primary problem or it can be associated with many other disorders. Some of them we're just figuring out. And so it's complicated. Because it crosses over many different disciplines. And probably the pathobiology is quite diverse. And then within pediatrics, it really brings up some special circumstances that really aren't reflected in the adult population. So I think we have to kind of readdress, reconsider some of the fundamental things that we think about with pulmonary hypertension. So for example, let's start with the definition. So the definition is a hemodynamic definition, even though it's a structural and functional disease of the pulmonary vasculature and ultimately the right heart. We have a hemodynamic definition, which is mean pulmonary arterial pressure of greater or equal to 25 millimeters of mercury at rest or 30 millimeters of mercury during exercise and a calculated pulmonary vascular resistance of three woods units or greater. So unfortunately, that definition may work reasonably well for the adult population. But there are several circumstances where it doesn't really adequately characterize the disease in the pediatric population. One most notably would be patients with single ventricular heart disease, where as you know, since they ultimately go on to have a passive pulmonary blood flow system, any modest elevation in pulmonary vascular resistance can have a profound clinical impact on their outcome and potential surgical candidacy.
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