14 Nov 2022

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Pulmonary Hypertension: Causes, Symptoms, and Treatment

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Academic level: College

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 Pulmonary hypertension (PH) is a life-threatening disease that occurs when the pulmonary arteries become clogged and narrowed. Patients with PH often have pulmonary arterial pressure greater than 25 mm Hg at rest (Orem, 2017). During exercise, this pressure tends to be higher than 30 mm Hg. The disease is usually characterized by a progressive and sustained increase in pulmonary vascular resistance, resulting in progressive ventricular failure and functional decline. Pathophysiological mechanisms that cause PH are –increased pulmonary venous pressure, which is cased disorders that affect the heart, especially the left side, and increased pulmonary vascular resistance, which is caused by pathogenic vasoconstriction ( Wrobel, Thompson, & Williams, 2012) . It can also be caused by the obliteration of the pulmonary vascular bed. 

PH mainly affects the small resistance pulmonary arteries. Plexiform arteriopathy is the pathognomonic lesion of PH (Orem, 2017). The body system involved in PH is the arteries and the right side of the heart. There are five types of PH –Group 1 Pulmonary Arterial Hypertension (PAH) caused by inherited genes, drugs, HIV infection, liver disease, and other conditions that affect the veins and blood vessels; Group 2 PH which is often associated with left heart disease; Group 3 HP, which is related to lung problems such as sleep apnea and interstitial lung disease; Group 4 PH, which is associated with blood clots in the lungs; and Group 5 PH which is triggered by other disorders such as polycythemia, vasculitis, and sarcoidosis (Orem, 2017). 

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The incidence and prevalence of PH in America are unknown. This can be as a result of the broad classification. It can also be a result of multiple aetiologies. PH is more frequently diagnosed in elderly patients. According to a study conducted by the National Institutes of Health (NIH), the mean age of diagnosis is between ( Orem, 2017). H owever, the disease can also be diagnosed in younger patients. 

Normal Anatomy of the Major Body System Affected 

The heart is composed of two parts, which are separated by the septum. Each side is further divided into two compartments –the upper compartment (atrium) and the lower compartment (ventricle). The right atrium receives deoxygenated blood coming back from the body. This blood is received through the vena cava. This blood is then passed to the right ventricle, which pumps the blood to the lungs. This blood is pumped by way of the pulmonary artery. The lung tissue is made of capillaries, and the pulmonary artery branches into these capillaries. In the capillaries, the blood becomes oxygenated. The capillaries braches into pulmonary veins and these veins pump the blood, which has been oxygenated back to the heart. This blood enters into the left atrium, which is then pumped into the left ventricle. The left ventricle, in turn, pumps the blood to the aorta. Once the oxygenated blood reaches the aorta, it can be pumped to the whole body. 

Normal Physiology of the Major Body System Affected 

PH is a result of inflammation and changes in the cells that line the pulmonary artery ( Wrobel, Thompson, & Williams, 2012) . Inflammation often reduces the diameter of blood vessels. This condition tends to restrict the flow of blood. These arterial changes make it difficult for the heart to supply blood. More to this is that the damaged blood vessels have high resistance to blood flow. As a result, there is an increase in blood pressure in the pulmonary arteries, which eventually makes the right ventricle to enlarge and to become weak. 

Mechanism of Pathophysiology 

There are two mechanisms of PH pathophysiology, which are "increased pulmonary vascular resistance and increased venous pressure” ( Wrobel, Thompson, & Williams, 2012) . Increased pulmonary vascular resistance is usually caused by pathologic vasoconstriction. It can also be caused by the obliteration of the pulmonary vascular walls or both ( Wrobel, Thompson, & Williams, 2012) . Numerous factors lead to vascular wall remodeling that is associated with PH. Some of these factors include chronic inflammation, hypertrophy, and vasoconstriction endothelial. To add to this, vasoconstriction is associated with thromboxane. 

Vascular obstruction tends to increase pulmonary pressure. It is also responsible for endothelium injuries that activate coagulation. Because of this condition, PH gets worse, which is impaired by "a decrease in tissue plasminogen activator activity that occurs as a consequence of platelet dysfunction, plasminogen activator inhibitor type 1, and fibrinopeptide” ( ( Wrobel, Thompson, & Williams, 2012) ). 

The other mechanism of PH pathophysiology is increased pulmonary venous pressure. This is usually caused by a medical condition that tends to harm the left side of the heart ( Wrobel, Thompson, & Williams, 2012) . This often increases pressure in the left heart ventricle. As a result, excess pressure is created, which, in turn, makes the pulmonary arteries to gain high pressure. This condition often leads to acute injuries in the alveolar-capillary wall and subsequent edema ( Wrobel, Thompson, & Williams, 2012) . To add to this, excessive stress can compromise the function of the lung as it can result in irreversible thickening of the walls of the alveolar-capillary membrane. 

Prevention 

Generally, PH can be prevented by making healthy lifestyle changes. Some of these lifestyle changes include eating healthy foods, exercising regularly, cutting down salt, and quitting smoking. These preventive measures help lower the chances of developing PH. Other measures that can help prevent this order include avoiding pregnancy and birth control pills and avoiding situations that can excessively lower blood pressure, such as sitting in a hot tube. 

Treatment 

PH cannot be treated, but physicians or healthcare professionals can patients manage this condition. The treatment options that are available for PH help improve the symptoms. It also helps slow the progress of PH. PH is commonly treated with medications, which include blood vessel dilators (vasodilators), endothelin receptor antagonists, sildenafil and tadalafil, high-dose calcium channel blockers, soluble guanylate cyclase (SCG) stimulator, anticoagulants, digoxin, and diuretics (Hoeper, Ghofrani, Grunig, Klose, Olschewski, & Rosenkranz, 2017). If medication does not control a patient's HP, surgeries can be done. Some of the surgeries done include atrial septostomy and lung transplantation. 

Clinical Relevance 

Phis clinically significant because the condition or disease is associated with increased symptoms. More to this is that PH is associated with a higher risk of death. This is true for all types of PH. The life expectancy of a patient with PAH has increased over the past 30 years (Hoeper, Ghofrani, Grunig, Klose, Olschewski, & Rosenkranz, 2017). The survival rate of patients with PH is quite higher now as when compared to 30 years ago. This is due to the introduction of effective treatment options. 

Conclusion 

PH is a rare and very life-threatening disease if not treated. Pathophysiological mechanisms that cause PH are –increased pulmonary venous pressure and increased pulmonary vascular resistance. Generally, PH can be prevented by making healthy lifestyle changes. There are also numerous treatment options and surgeries for PH. The treatment options that are available for PH help improve the symptoms of the condition as well as slow the progress of PH. 

References 

Hoeper, M., Ghofrani, H.m Grunig, E., Klose, H., Olschewski, H., & Rosenkranz, S. (2017). Pulmonary hypertension. Deutsches Arzteblatt Internation. Vol. 114 (5): 73-84. https://dx.doi.org/10.3238%2Farztebl.2017.0073

Orem, C. (2017). Epidemiology of pulmonary hypertension in the elderly. Journal of Geriatric Cardiology. Vol. 14 (1): 11-16. https://dx.doi.org/10.11909%2Fj.issn.1671-5411.2017.01.001

Wrobel, J. P., Thompson, B. R., & Williams, T. J. (2012). Mechanisms of pulmonary hypertension in chronic obstructive pulmonary disease: a pathophysiologic review.  The Journal of Heart and Lung Transplantation 31 (6), 557-564 

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StudyBounty. (2023, September 14). Pulmonary Hypertension: Causes, Symptoms, and Treatment.
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