Pulmonary Hypertension: Classification: Class 1 & 2

by Carlo Raj, MD

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    00:00 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.

    00:19 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.

    02:30 Okay, now, important causes include drugs such as amphetamines, cocaine, connective tissue disorders, HIV, portal hypertension and congenital heart disease and schistosomiasis.

    02:44 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.

    03:23 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.

    03:35 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.

    03:51 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.

    04:01 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?

    About the Lecture

    The lecture Pulmonary Hypertension: Classification: Class 1 & 2 by Carlo Raj, MD is from the course Disorders of the pulmonary circulation and the respiratory regulation.

    Included Quiz Questions

    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

    Author of lecture Pulmonary Hypertension: Classification: Class 1 & 2

     Carlo Raj, MD

    Carlo Raj, MD

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