Pulmonary Hypertension

by Carlo Raj, MD

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    00:02 The topic now is Pulmonary Hypertension. As simple as it may seem, it's the fact that you have to dive into quite a bit of detail and the reason for that is because there has been so much research on this over the years because of sequelae of right-sided heart failure, cor pulmonale and death, that now, at this point, we know how to, first, identify normal, completely analyze the pathology through WHO classifications and every single class you will have to know, there's four of them and then management.

    00:34 To begin with, Pulmonary Hypertension, defined as, well, the big one here that you want to know is greater than 25mmHg at rest. So, how would you go about, perhaps, measuring this? How about a right heart catheterization? So, here, when you catheterize the right side and you get from the right ventricle and moved up to you pulmonary artery. Alright, can you picture that? Good. Right ventricle, you have a pressure of approximately 2, very passive, but then you start getting to pulmonary artery, the pressure increases to approximately 10, but at rest, if you find that the pulmonary arterial pressure is greater than 25, guaranteed, your patient has Pulmonary Hypertension. But what caused this? Well, let's continue.

    01:26 The Pulmonary Hypertension may result in arteriosclerosis. What does that mean? Imagine when there is an increase in tension, then what's going to happen? Is this arteriolar is this an arteriole? That is your first question, isn't it? Always. Whenever you deal with blood vessels, you want to try to break it up into the arteries and arterioles and then that will then give you the respected diagnosis. Here, it is arteriosclerosis, most likely, hyaline type. Then you have medial hypertrophy, intima fibrosis of pulmonary arteries and by that we mean that, here is my intima, there is my media and so, those are two major layers of the blood vessels that are being affected. There will be medial hypertrophy and intimal fibrosis, causing what, please? Increased narrowing of the pulmonary arteries. Now, the clinical course includes severe respiratory distress. Whenever there is problem with the pulmonary artery and there is increased resistance or pressure you are then going to feel this in the right ventricle resulting in right ventricle hypertrophy (RVH) and unfortunately, getting into severe failure, cor pulmonale. Now, this is what I was referring to earlier, with the WHO classification of Pulmonary Hypertension. I am going to give you a brief description for every single class that you absolutely must know because the way that you approach this or clinical vignettes, I will show you, will be a class by class.

    02:57 Begin with Class I. Now, we will begin, chronological order, by Class I. Clinically however, you will begin this a little bit different. Why? Keep it in mind. If it is familial, very difficult to sometime figure this out immediately, isn't it? So, it is easier to actually perform certain tests that are a lot faster to rule things out, Class II, III and IV, so then you would highly suspect Class I. Let’s put it that way. Once more, this is familial. What does that mean to you? Once upon a time, known as your primary Pulmonary Hypertension, get away from that, idiopathic pulmonary arterial hypertension is a much better descriptive name. Majority of these will be familial. Okay. That means that there is no secondary effect. There is no hypoxia in which there is there's pulmonary vasoconstriction. There is no heart disease causing pulmonary arterial hypertension and there is no thromboembolic episode, chronically, resulting in increased pulmonary hypertension. Right? This is familial. So, it is actually faster and more effective to rule out, and we will go through each one, I,II,III and IV, and then to see as to whether or not Type 1, in fact, is your proper diagnosis. Now, what do you want to know about familial? Oh, it's an inactivation mutation of a particular gene called your bone morphogenic protein-receptor-2. Know the entire name. I'm sorry, you must. The abbreviation is BMPR2. R is Receptor. BMP is bone morphogenic protein. What does it do? It normally inhibits the vascular smooth muscle proliferation and so, therefore if you have such a mutation, where you inactivate the gene, which normally controls the proliferation, where am I? Blood vessel. And so therefore, if you remove this suppressor, you have increased proliferation, end up having pulmonary arterial hypertension. Prognosis, poor.

    05:08 Okay, now, important causes include drugs such as amphetamines, cocaine, connective tissue disorders, HIV, portal hypertension and congenital heart disease and schistosomiasis.

    05:21 If I were you, I would take a look at each one of these differentials. How do you rule them out? Your patient is an addict. Your patient has scleroderma. Do you remember a topic when we had scleroderma and it was the continuum of fibrosis that you are paying attention to and it’s a fact that we had crust and we had systemic type and so, therefore, if it is fibrosis that you are dealing with, then it is the fact that you are, at first, having non-specific changes and then your usual interstitial pneumonitis which we as pathologist, are then going to describe it as. Are we clear? Okay.

    06:01 HIV, immuno-compromised. How do you rule out something that’s causing liver injury? Check out your liver function test. Are we clear? That’s important. Rule things out.

    06:13 Congenital heart disease. What kind of a test might you want to perform on a patient to rule out congenital heart disease? Good. A Cardiac MRI or a Cardiac Magnetic Resonance test. Clear? Schistosomiasis. Well, that might be history.

    06:29 Maybe patient came from North Africa, Egypt specifically. That type of region where the patient might have been exposed to Schistosoma. That’s Class I.

    06:39 Move on to Class II, please. Now Class II, the way that I have thought about this and the way that maybe might help you, Class II, is there any way that you could possibly think of your heart having two sides? Would that perhaps help you? Okay. The reason I say that is, Type 2, or Class II, excuse me, type of Pulmonary Hypertension is going to be trouble in your pulmonary blood vessels secondary to the heart, secondary to the heart. Are we clear? So, pay attention to that and so, if it helps you to think of the heart as having two sides. And let’s say that you have right ventricle hypertrophy and you have to start on the left side. So, if you have left-sided heart failure, then you might back up into pulmonary veins. Right? You further back up into pulmonary capillary, pulmonary artery. So, therefore, here, the Pulmonary Hypertension was due to a left ventricle heart disease. That’s Class II. How would you rule this out? Maybe something as simple as E & E. What are these? Echocardiogram, EKG. Alrighty? Okay, let’s go on to talk about Class III. Now, Class III type of Pulmonary Hypertension and all of these, ladies and gentlemen, you really must know in detail. Okay. So, I am not wasting your time. With Class III, it is actual lung disease that took place primarily.

    08:03 In Type 1, the majority of a more familial, but then you also had drugs and other habits or diseases, but then here, if it’s Type 3, it is primary lung parenchymal disease of all of which COPD would be the most common. Okay. Now, along with COPD, say that your patient is suffering from hypoxia, now, what’s the state of your pulmonary blood vessels? It is called, remember, one of the big exceptions, when there is hypoxia in which blood vessels constrict would be hypoxic vasoconstriction and nowadays, in current day practice, your patients very well might be obese and so, therefore, you are thinking about obstructive sleep apnea, and then, of course, high-altitude residence. Here once again, because of low barometric pressure, thus resulting in decreased FiO2, you find your patient having hypoxic vasoconstriction. That’s Class III. Then we go into Class IV. Class IV, if it helps you any further, you need to remember, this is a thromboembolic disease causing a Pulmonary Hypertension. So, we had a discussion about PE and all the different ways in which you might then develop a PE. The most common of which would be your DVT, right? But, keep in mind though that you can also have embolization from different sources. We talked about the amniotic fluid emboli, we talked about the gas emboli, the fat emboli, all depending as to the situation. Anyhow, so there is a thromboembolic disorder and what I was trying to say is, if you want to perhaps think of four and how would you say that perhaps in Latin, it would be tetra. Alright, it's tetra. Think of it as being thromboembolic. So, give yourself whatever you need to do to distinguish Class I through Class IV. Try to do it now and get an understanding and keep coming back and keep applying test. So, let's do that now, once again.

    10:00 We ruled out our heart issue if you had maybe normal EKG and normal Echo. That’s good enough for you right now. If it’s Class III, well, you take a look at the chest X-ray and say that this was pneumonia, alright, or it was COPD, you would expect there to be quite a bit of marking on your X-ray, wouldn’t you? So, this would then obviously tell you that I have a serious problem with my lung primarily and therefore causing vasoconstriction or perhaps increased Pulmonary Hypertension. And with Class IV, you tell me, what would be effective in terms of ruling out, perhaps, something like a DVT and such? You are thinking about a VQ scan. okay. A VQ Scan. Remember, you don’t want to get into a PE. If you get into a PE then it will be a spiral CT, right? So, a VQ scan will perhaps help you for ruling out, effectively, a chronic thromboembolic disease. Now, there are multifactorial.

    10:56 Some would say that this is a Class V, whatever. Here you go. Hematologic, systemic, metabolic disorders. These are the other ones that really don’t fit into Class I through Class IV. And the reason that this is important is now the approach, the approach of Pulmonary Hypertension. The Pulmonary Hypertension, it's much easier for you to rule out effectively Class II. What is that? Where is my problem? How is that causing Pulmonary Hypertension? The heart. Secondary Pulmonary Hypertension. Next. Class III. How would you effectively rule that out? A relatively unremarkable chest X-ray. And then Class IV, maybe a normal VQ scan. You rule out II, III and IV. What are you really left with right now? Good. Class I. And so, then you start thinking about your genes such as BMPR2, Bone Morphogenic Protein-2, maybe drugs and so on and so forth. Are we clear? So, this is “Pulmonary Hypertension”, Classes I through IV. I would be very, very familiar with that table.

    About the Lecture

    The lecture Pulmonary Hypertension by Carlo Raj, MD is from the course Disorders of the pulmonary circulation and the respiratory regulation. It contains the following chapters:

    • Pulmonary Hypertension
    • Classification: Class 1 & 2
    • Classification: Class 3 & 4

    Included Quiz Questions

    1. 10-14 mmHg
    2. 25-30 mmHg
    3. 0-10 mmHg
    4. 14-30 mmHg
    5. 10-30 mmHg
    1. cor pulmonale
    2. right ventricular hypertrophy
    3. arteriosclerosis
    4. COPD
    5. right atrial enlargement
    1. BMPR2
    2. DMD
    3. DHCR7
    4. ADA
    5. CFTR
    1. Loss of inhibition of vascular smooth muscle proliferation.
    2. Loss of elastase enzyme inhibition.
    3. Loss of elastase binding and inactivation.
    4. Inappropriate activation of inflammatory mediators.
    5. Inappropriate activation of serine proteases.
    1. Thromboembolic disease
    2. Systemic Scleroderma
    3. HIV
    4. Stimulant abuse
    5. Schistosomiasis
    1. Echocardiogram
    2. Pulmonary function testing
    3. Pulmonary angiography
    4. Spiral CT
    5. CXR
    1. Polysomnography
    2. Echocardiogram
    3. Genetic screening
    4. Cardiac MRI
    5. D-dimer
    1. Chronic thromboembolic disease
    2. Lung parenchymal injury
    3. Substance abuse
    4. Hypoxic vasoconstriction
    5. Left sided heart disease
    1. Class III
    2. Class I
    3. Class V
    4. Class II
    5. Class IV

    Author of lecture Pulmonary Hypertension

     Carlo Raj, MD

    Carlo Raj, MD

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