What Is Pulmonary Hypertension?
To better understand chronic pulmonary thromboembolic hypertension, it is important to understand what it is. When the pressure in the blood vessels connecting the heart and lungs is too high, the condition is known as pulmonary hypertension. Pulmonary hypertension specifically affects the right side of the heart and the lung arteries. Pulmonary hypertension can occur due to several causes. Symptoms of pulmonary hypertension include shortness of breath or dyspnea, pain in the chest, tiredness, rapid heartbeat, and swelling or edema of the legs.
What Is Chronic Thromboembolic Pulmonary Hypertension?
Chronic thromboembolic pulmonary hypertension (CTEPH) is a form of pulmonary hypertension. CTEPH is a progressive complication of pulmonary hypertension. Blood clots in the lungs that do not dissolve lead to CTEPH. These clots result in tissues like scar tissue, which narrows or obstructs the tiny blood vessels of the lungs. Pulmonary pressure rises, leading to pulmonary hypertension, as the blood vessels become increasingly constricted and narrow. This implies that to pump blood via the right side of the heart; it must exert higher pressure. CTEPH is a possible complication of pulmonary embolism and a crucial cause of long-term pulmonary hypertension, which may lead to heart failure (right side).
What Causes Chronic Thromboembolic Pulmonary Hypertension?
When small blood clots in the lung's blood vessels fail to disintegrate, CTEPH develops. Blood vessels become constricted due to the development of scar-like tissue, and the pressure increases as the constriction become more severe. Moreover, certain risk factors can contribute to the development of CTEPH. Common risk factors associated with chronic thromboembolic pulmonary hypertension include the following:
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Obesity.
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Smoking.
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Cancer.
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High blood pressure or hypertension.
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Prolonged use of birth control pills.
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Old age.
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Individuals who have undergone surgery.
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Blood groups other than O.
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Osteomyelitis and other bone infections.
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Inflammatory bowel disease.
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Splenectomy (surgical removal of the spleen).
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Infection of heart tissues due to pacemakers.
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Individuals who are undergoing thyroid hormone replacement therapy.
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History of blood or clotting disorder.
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Family history of clotting disorders.
Is Chronic Thromboembolic Pulmonary Hypertension a Common Condition?
Chronic thromboembolic pulmonary hypertension is a rare condition. In the United States, approximately 5,000 new cases are reported each year. However, the condition is not often accurately diagnosed, so the number of reported cases may be underestimated. About 1 to 5 percent of people with pulmonary embolism tend to develop CTEPH.
What Are the Symptoms of Chronic Thromboembolic Pulmonary Hypertension?
The symptoms of CTEPH are similar to symptoms of other conditions like asthma, emphysema, chronic obstructive pulmonary disease (COPD), and heart failure, which is why CTEPH can be challenging to diagnose.
Symptoms of CTEPH include the following:
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Shortness of breath, especially while climbing stairs or doing other strenuous physical activity.
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Fatigue and tiredness.
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Edema of the legs.
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Tightness in the chest.
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Feeling dizzy.
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Fainting and syncope.
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Heart palpitations and increased heart rate.
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Blood in cough, but this rarely occurs.
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Cyanosis or bluish discoloration of fingers and toes due to lack of oxygen.
How Is Chronic Thromboembolic Pulmonary Hypertension Diagnosed?
CTEPH can be diagnosed with the help of the following tests:
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Ventilation-Perfusion (V/Q) Scan: This examination evaluates how well air and blood flow into the lungs. This scan is the best diagnostic procedure to confirm CTEPH. The scan generates images that the medical staff examines to determine whether the patient has blood clots in their lungs.
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Echocardiogram: This test is like an ultrasound of the heart, a non-invasive procedure. An echocardiogram enables the doctor to see the anatomy and functioning of the heart. This test can analyze certain signs of pulmonary hypertension in most patients with the condition.
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Lung Function Test: A lung function test measures the patient's ability to hold air and the rate at which air enters and exits the lungs. The doctor can diagnose any abnormality in the tissues of the lungs.
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Right-Sided Heart Catheterization: When other tests suggest a diagnosis of pulmonary hypertension, the doctor may suggest a right-sided heart catheterization. This test determines the type of pulmonary hypertension. During this test, a catheter (a small tube) is inserted into a major vein in the neck and arm. The tube is then advanced to the right side of the heart and then into the blood vessels that link to the lung. The doctor then evaluates the pressure in different parts of the heart and the pulmonary artery.
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Pulmonary Angiogram: During this test, a dye or contrast is injected into the veins, and X-rays are taken of the lungs' blood vessels. With pulmonary angiography, the doctor can locate blood clots and blocked blood vessels in the lungs.
How Is Chronic Thromboembolic Pulmonary Hypertension Treated?
Chronic thromboembolic pulmonary hypertension can be treated in the following ways:
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Surgery: Surgery known as pulmonary thromboendarterectomy (PTE) or pulmonary endarterectomy is the best and most preferred treatment for CTEPH. This procedure is likely to cure the condition in about 90 % of the patients. Before the PTE treatment, the patients are placed on a heart-lung bypass apparatus to help the other organs receive blood flow. The surgeon will then stop the heart while clearing out all clots from the arteries of the lungs. Moreover, a lung transplant can be done in severe cases where all other treatments fail to improve the condition.
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Medication: Patients who cannot undergo surgery or continue to have pulmonary hypertension following surgery should be treated with medications. These medications may be prescribed via online prescriptions. One can consider getting an best online prescription refill incase of pre-existing medications if they are unable to have in-person doctor visits.
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The only drug approved by the United States Food and Drug Administration is Ronociguat. To prevent new blood clots from developing, all CTEPH patients—including those who have undergone surgery should take blood thinners (anticoagulants) for the rest of their lives.
Conclusion:
Chronic thromboembolic pulmonary embolism (CTEPH) is an uncommon and serious type of pulmonary hypertension. The condition is a complication of pulmonary embolism and presents a variety of symptoms similar to several other conditions. Surgery can treat CTEPH in most patients. Drugs and other treatments are available for patients who cannot undergo surgery. Patients with CTEPH can die within five years if they are not treated. Therefore, it is essential to diagnose and treat CTEPH. However, with proper treatment and care, CTEPH has a favorable prognosis.
