OverviewPulmonary hypertension is a type of high blood pressure that affects the arteries in the lungs and the right side of the heart. Show
In one form of pulmonary hypertension, called pulmonary arterial hypertension (PAH), blood vessels in the lungs are narrowed, blocked or destroyed. The damage slows blood flow through the lungs, and blood pressure in the lung arteries rises. The heart must work harder to pump blood through the lungs. The extra effort eventually causes the heart muscle to become weak and fail. In some people, pulmonary hypertension slowly gets worse and can be life-threatening. Although there's no cure for some types of pulmonary hypertension, treatment can help reduce symptoms and improve quality of life. SymptomsThe signs and symptoms of pulmonary hypertension develop slowly. You may not notice them for months or even years. Symptoms get worse as the disease progresses. Pulmonary hypertension signs and symptoms include:
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information with other information we have about you. If you are a Mayo Clinic patient, this could include protected health information. If we combine this information with your protected health information, we will treat all of that information as protected health information and will only use or disclose that information as set forth in our notice of privacy practices. You may opt-out of email communications at any time by clicking on the unsubscribe link in the e-mail. CausesThe typical heart has two upper chambers (atria) and two lower chambers (ventricles). Each time blood passes through the heart, the lower right chamber (right ventricle) pumps blood to the lungs through a large blood vessel (pulmonary artery). In the lungs, the blood releases carbon dioxide and picks up oxygen. The blood typically flows easily through blood vessels in the lungs (pulmonary arteries, capillaries and veins) to the left side of the heart. However, changes in the cells that line the pulmonary arteries can cause the walls of the arteries to become stiff, swollen and thick. These changes may slow down or block blood flow through the lungs, causing pulmonary hypertension. Pulmonary hypertension is classified into five groups, depending on the cause. Group 1: Pulmonary arterial hypertension (PAH)Causes include:
Group 2: Pulmonary hypertension caused by left-sided heart diseaseCauses include:
Group 3: Pulmonary hypertension caused by lung diseaseCauses include:
Group 4: Pulmonary hypertension caused by chronic blood clotsCauses include:
Group 5: Pulmonary hypertension triggered by other health conditionsCauses include:
Eisenmenger syndrome and pulmonary hypertensionEisenmenger syndrome is a type of congenital heart disease that causes pulmonary hypertension. It's most commonly caused by a large hole in the heart between the two lower heart chambers (ventricles), called a ventricular septal defect. This hole in the heart causes blood to flow incorrectly in the heart. Oxygen-carrying blood (red blood) mixes with oxygen-poor blood (blue blood). The blood then returns to the lungs — instead of going to the rest of the body — increasing the pressure in the pulmonary arteries and causing pulmonary hypertension. Risk factorsPulmonary hypertension is more often diagnosed in people ages 30 to 60. Growing older can increase the risk of developing Group 1 pulmonary hypertension, called pulmonary arterial hypertension (PAH). However, PAH from an unknown cause (idiopathic PAH) is more common in younger adults. Other things that can raise the risk of pulmonary hypertension include:
ComplicationsPotential complications of pulmonary hypertension include:
How is risk assessed in patients with pulmonary hypertension (PAH)?Approaches for assessing risk in PAH patients include the use of risk variables, as recommended in the 2015 European Society of Cardiology (ESC)/European Respiratory Society (ERS) pulmonary hypertension (PH) guidelines, and the application of risk equations and scores, such as the French registry risk equation and the REVEAL registry risk score.
How do medications affect the kidneys in patients with PAH?However, all these medications also put increased stress on your kidneys. In PAH, the kidneys are already under stress from impaired right heart function. Taking these medications promotes fluid retention and can lead to abrupt worsening in kidney function and increased shortness of breath over a short period of time.
Why does pulmonary arterial hypertension (PAH) occur?In PAH, this increased pressure in the vessels is caused by obstruction in the small arteries in the lung for a variety of reasons. PAH occurs when the very small arteries throughout the lungs narrow in diameter, which increases the resistance to blood flow through the lungs.
What changes in the blood vessels cause pulmonary hypertension?However, changes in the cells that line the pulmonary arteries can cause the walls of the arteries to become stiff, swollen and thick. These changes may slow down or block blood flow through the lungs, causing pulmonary hypertension.
Which medication exposure in a patient increased the risk for primary pulmonary arterial hypertension?According to the current classification PAH can be associated with exposure to certain drugs or toxins, particularly to appetite suppressant intake drugs, such as aminorex, fenfluramine derivatives and benfluorex.
Which people are considered at higher risk for inheriting pulmonary arterial hypertension PAH )?Pulmonary arterial hypertension is more frequent in women between the ages of 30 and 60. Since there is an inherited form of PAH, a family history of the disease may put you at increased risk.
Which class of drugs provides the best specific dilation of pulmonary blood vessels in a patient?Blood vessel dilators (vasodilators).
This type of medicine relaxes and opens narrowed blood vessels, improving blood flow. Vasodilators may be taken by mouth, inhaled, injected or given by IV infusion. A commonly prescribed vasodilator for pulmonary hypertension is epoprostenol (Flolan, Veletri).
Which diagnostic test is used to determine the diffusion capacity in a patient with pulmonary arterial hypertension?Pulmonary Function Testing
Pulmonary function tests (ie, spirometry and diffusing capacity for carbon monoxide) should be performed in patients with pulmonary hypertension to exclude an underlying pulmonary disorder.
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