Pulmonary hypertension is an increase in the pressure in the pulmonary circulation due to vasoconstriction or arteriopathy involving the pulmonary vasculature.
Pulmonary Arterial Hypertension (PAH) Farid Taymouri,MD Rheumatology Private Practice Tehran-IRAN
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What Is PAH?
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What is PAH ? And how is it different than Pulmonary Hypertension?
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Pulmonary hypertension is an increase in the pressure in the pulmonary circulation due to vasoconstriction or arteriopathy involving the pulmonary vasculature. In the pulmonary circulation, the pulmonary arterial pressure is usually approximately 25/10 mm Hg. Mean pulmonary artery pressure is in the range of 12 to 14 mm Hg. When it rises above 25 mm Hg, it is defined hemodynamically as PAH.
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How is PH classified?
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Before 2003, PH patients were divided into those with primary pulmonary hypertension,or PPH, and those with secondary pulmonary hypertension,or SPH.
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In 2003, experts realized that the term SPH did not properly separate the different types of PH that exist,and this made it very difficult to classify and manage PH patients.
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In 2003, at a world meeting held in Venice, Italy PH experts changed the PH terminology. Farid Taymouri,MD
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In 2008, in Dana Point,Calfornia recent classification system occurred, and is known as the Dana Point Classification. Farid Taymouri,MD
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Briefly, all patients with PAH fall into the category called WHO Group 1,which includes previously-named group PPH, and now known as IPAH (Idiopathic Pulmonary Hypertension).
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Group 1, is PAH, and within PAH there are different subgroups. There is a group of patients who have idiopathic PAH, and there are patients with associated PAH. Associated PAH can be due to a number of different disorders, specifically Connective Tissue Diseases such as scleroderma, which is most common, but lupus and dermatomyositis can also cause PH. Congenital Heart Disease is another cause of PAH; atrial septal defects, ventricular septal defects, and anything leading to left to right shunting in Eisenmenger physiology can also result in pulmonary hypertension. A third subgroup of patients who fall within group 1 PAH are those with liver disease, such as portopulmonary hypertension. Human immunodeficiency virus (HIV) is another cause of pulmonary hypertension also classified under WHO group 1 PAH. Approximately 0.5% of patients with HIV will develop PAH at some point in their disease course, so that is certainly something to be aware of. There is also a heritable form of PH, in which patients have a family history of pulmonary hypertension. Of all the patients with idiopathic PAH, approximately 5% to 10% of them will have the heritable form, meaning someone in their family might previously have had PAH.
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WHO group 2 PAH have pulmonary hypertension secondary to heart disease (regardless of the form of heart disease),
valvular such as mitral stenosis or atrial stenosis, or various cardiomyopathies. Patients with atrial myxomas and other rare conditions affecting the left side of the heart can lead to pulmonary hypertension. Probably the most prevalent conditions affecting the left side of the heart that lead to pulmonary hypertension is diastolic dysfunction or heart failure with normal ejection fraction(HFNEF). These patients mostly referred to pulmonary hypertension centers, and are very difficult to diagnose, on the contrary to those with systolic heart failure, that ejection fractions are frankly in the low range and easily identified on echocardiogram. Farid Taymouri,MD
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WHO group 3 PAH patients have pulmonary hypertension due to any form of lung disease. Patients with conditions such as interstitial lung disease, idiopathic pulmonary fibrosis, or COPD, would be classified as having group 3 pulmonary hypertension. Any patient with any form of diffuse parenchymal lung disease that advances over time can develop secondary pulmonary hypertension. Another important cause of pulmonary hypertension that would classify a patient in group 3 is obstructive sleep apnea.
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Group 4 refers to patients with Chronic ThromboEmbolic Pulmonary Hypertension (CTEPH). These patients have had a thromboembolic event at some point in the past, and develop chronic pulmonary hypertension from the thromboembolic event that fails to resolve. patients who have had pulmonary emboli in the past, approximately 1% to 3% of them will develop CTEPH.
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WHO group 5 are patients who have miscellaneous causes of pulmonary hypertension, including rare entities such as Sarcoidosis and Lymphangioleiomyomatosis. Also fairly common conditions, such as Chronic Renal Failure, can be classified under group 5, pulmonary hypertension.
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Prevalence of PAH? PAH is a rare condition. prevalence is anywhere from approximately 15 to 60 per million population. The incidence, the annual occurrence of pulmonary hypertension (new diagnoses), is in the range of approximately 3 to 7 cases per million.
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Clinical Picture What does the typical patient with PAH look like?
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The patient may present with nonspecific symptoms of:
fatigue weakness shortness of breath
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It is easy to make other diagnoses, which are more common, such as asthma, chronic fatigue syndrome, or thyroid issues. Because PAH is a rare disease and because the disease tends to present nonspecifically, in many instances the patient will go undiagnosed for several years.
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The diagnosis of PAH is challenging. Before the diagnosis of PAH, a number of different tests need to be performed.
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What is important in the context of making
a diagnosis of PAH is to rule out pulmonary hypertension due to groups 2, 3, 4, and 5
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Within group 1 PAH Connective Tissue Disease serologies to exclude CTD HIV status Liver function to ensure that there is no occult liver disease
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To rule out heart disease in Group 2, Generally echocardiography is a reliable tool. Echocardiography is a very good way to screen for pulmonary hypertension, It is also one of the diagnostic tests that we use to rule out things such as shunt physiology as a cause of Eisenmenger physiology, resulting in pulmonary hypertension. It is also helpful when looking at the left side of the heart to diagnosis any form of left-sided heart disease, valvular disease, systolic dysfunction, or diastolic dysfunction.
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Importance of a good history in making the diagnosis: A good history is very important We often obtain very valuable information from a patient's history We should always start with a good history and physical examination Farid Taymouri,MD
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In Group 3, It is necessary to rule out any form of Lung disease Pulmonary Function Testing(PFT) Computed Tomography (CT) scan of the chest (The CT scan of the chest also enables us to exclude other conditions that are categorized under group 5, such as sarcoidosis.)
can be very helpful to identify occult interstitial lung disease and sometimes changes consistent with COPD Farid Taymouri,MD
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PAH in the Scleroderma screening process in scleroderma patients in order to diagnose pulmonary arterial hypertension (PAH) earlier and with greater accuracy
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Scleroderma lung disease is currently the most common cause of scleroderma-related deaths,
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Right Heart Cathetrization: Right heart catheterization (RHC) is required for a diagnosis of pulmonary arterial hypertension (PAH). It is the gold standard for assessing the hemodynamics of pulmonary hypertension, assessing disease severity, and determining prognosis and response to therapy .
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Diagnostic approach to pulmonary hypertension.
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Thank You
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