Pulmonary Arterial Hypertension is similar to cancer and inflammation (1)
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Pulmonary Arterial Hypertension is similar to cancer and inflammation (1)

September 9, 2019

– What is pulmonary arterial hypertension? How to recognize its symptoms?
Dr. Anton Titov MD What causes pulmonary arterial hypertension? – The definition of pulmonary arterial hypertension
– in a way I wish we’d never called it that, because all it really implies that there is
an increase in pressure. But when you get down to the disease state
itself, pressure is a sign of the disease, it’s not the disease. The disease is really a pathologic process
that results in thickening and stiffening of the blood vessels inside the lung. And as those vessels get stiffer, they drive
up resistance to flow, which ultimately drives up pressure. But it’s really the pathologic change that
results in that increased stiffness, increased resistance, that imparts the pulmonary arterial
hypertension disease state. The difficult thing about the disease and
all aspects of pulmonary vascular disease is that patients generally present simply
with shortness of breath. And it’s usually shortness of breath that
is with exertion, not at rest. So if you think about shortness of breath,
it’s a very common complaint and it’s not specific to any disease. – When people call the disease pulmonary arterial
hypertension, it’s calling it by its signs, but in reality the causes of the disease are
somewhere else. – We don’t know what causes Pulmonary arterial
hypertension. But there are a host of associated disease
states. I think the important things that we’ve learned
over the past 15 – 20 years is that what happens to cause that change. There’s abnormal growth or proliferation of
cells – almost as in a neoplastic process. You see abnormal growth of smooth muscle cells
in certain regions of the vascular bed, and you’ll see abnormal growth of endothelial
cells and endothelial-like cells in different regions of the lung vascular bed. There’s also a significant inflammatory component,
where we not only see increased inflammatory mediators that circulate, but also almost
an invasion of inflammatory cells [in pulmonary arterial hypertension]. And then there’s also proliferation of connective
tissue cells. So that we also see fibrotic change. So pulmonary arterial hypertension ends up
being a very complicated disease, much like cancer. But it had been long thought to be much more
vasoconstrictive process, which it’s not, because if it was, it would have been easy
to treat years and years ago. – It’s certainly not the first time when the
disease gets a name before their nature is understood, so then the name becomes somewhat
misleading. But it’s very interesting to know that it’s
a very complicated process that lies behind the what’s known as pulmonary arterial hypertension. – Exactly!

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