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Pulmonary Hypertension: Causes, Symptoms, Risk Factors, Diagnosis, Classification, Treatment and Complications

Sep 13, 2023

Pulmonary Hypertension: Causes, Symptoms, Risk Factors, Diagnosis, Classification, Treatment and Complications

Pulmonary hypertension is a type of excessive blood pressure that affects the arteries in the lungs and the right side of the heart.

Pulmonary arterial hypertension (PAH), one type of pulmonary hypertension, is characterized by the narrowing, obstruction, or destruction of blood vessels in the lungs. The damage slows blood flow to the lungs. Blood pressure in the lungs' arteries rises. The heart must push blood through the lungs with higher force. The heart muscle eventually becomes weak and fails due to the increased strain.

Some people's pulmonary hypertension, which worsens gradually, can be fatal. There is currently no known cure for pulmonary hypertension. However, there are treatments that can improve your quality of life, help you live longer, and make you feel better.

Causes Of Pulmonary Hypertension

The heart consists of two upper and two lower chambers. The lower right chamber pumps blood to the lungs each time blood passes through the heart. The pulmonary artery, a significant blood vessel, carries the blood.

The blood arteries in the lungs often provide easy blood flow to the left side of the heart. The pulmonary arteries, capillaries, and veins make up this group of blood vessels.

However, alterations in the lining cells of the pulmonary arteries can result in the arterial walls becoming constrictive, rigid, bloated, and thick. Pulmonary hypertension may result from these alterations that impede or stop blood flow through the lungs.

Depending on the cause, pulmonary hypertension is divided into five types.

Pulmonary arterial hypertension (PAH) group 1.

Some causes are:

  • Idiopathic pulmonary arterial hypertension is a condition with no recognized cause.
  • Pulmonary arterial hypertension that is heritable is caused by changes in a gene that are handed down via generations.
  • Using some prescription pharmaceuticals or illicit substances, such as methamphetamine.
  • A congenital heart defect is when a person has cardiac issues from birth.
  • Other medical disorders, such as lupus, scleroderma, and cirrhosis, a chronic liver diseases.

Group 2: Left-sided heart disease-related pulmonary hypertension

The most typical type of pulmonary hypertension is this one. some causes are:

  • Failing the left-sided heart.
  • Disease of the mitral or aortic valves on the left side of the heart.

Group 3: Lung disease-related pulmonary hypertension

Some causes are:

  • Pulmonary fibrosis.
  • Pulmonary illness with chronic obstruction.
  • Apneic sleep.
  • Long-term high-altitude exposure for those who may have a higher risk of developing pulmonary hypertension.

Group 4: Pulmonary hypertension brought on by persistent blood clots or pulmonary arterial obstructions

Some causes are:

  • Pulmonary emboli are persistent blood clots in the lungs.
  • Pulmonary artery-blocking tumors.

Group 5: Other medical disorders that cause pulmonary hypertension

Some causes are:

  • Blood diseases, such as essential thrombocythemia and polycythemia vera.
  • Diseases that cause inflammation, like sarcoidosis.
  • Abnormalities of the metabolism, such as glycogen storage disease.
  • kidney illness.

Pneumonia and the Eisenmenger syndrome

Pulmonary hypertension is a symptom of the congenital cardiac condition Eisenmenger syndrome. Unrepaired perforations between the heart chambers might cause it. A ventricular septal defect, a significant opening between the two lower heart chambers, is an illustration.

Blood flows in the heart improperly as a result of the hole. Blood with and without oxygen combines. Instead of traveling to the rest of the body, the blood then returns to the lungs. Pulmonary hypertension is the result of increased blood flow and pressure in the pulmonary arteries.

Symptoms Of Pulmonary Hypertension

Pulmonary hypertension symptoms appear gradually. They might go months or even years before you notice them. Symptoms get worse as the disease progresses.

Symptoms of pulmonary hypertension include:

  • Breathlessness, first when exercising and then later while even lying still.
  • Due to insufficient oxygen levels, the skin may appear blue or gray. These modifications could be more or less obvious depending on the color of your skin.
  • Pressure or soreness in the chest.
  • Dizziness or episodes of fainting.
  • Fast heartbeat or rapid pulse.
  • Fatigue.
  • Swelling in the legs, belly, and ankles.

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Risk Factors Of Pulmonary Hypertension

The typical age range for pulmonary hypertension diagnosis is between 30 and 60. The likelihood of having Group 1 pulmonary hypertension, also known as pulmonary arterial hypertension (PAH), can rise with age. The prevalence of PAH in younger adults is higher.

In addition, the following factors can increase the risk of pulmonary hypertension:

  • A medical issue running through the family.
  • Being overweight.
  • Smoking.
  • Abnormalities of blood clotting or a family history of pulmonary blood clots.
  • Asbestos exposure.
  • A birth abnormality of the heart is known as a congenital heart defect.
  • Residing in an altitude.
  • The consumption of specific substances, such as some weight-loss medications and illicit drugs like cocaine or methamphetamine.

Diagnosis Of Pulmonary Hypertension

Because pulmonary hypertension is rarely discovered through a standard physical examination, it can be challenging to detect early. The signs of pulmonary hypertension are similar to other heart and lung disorders, even when it is more advanced.

A medical practitioner will evaluate you and inquire about your symptoms to determine if you have pulmonary hypertension. 


The following tests may be used to identify pulmonary hypertension:

  • Blood test. Blood testing might reveal symptoms of problems or assist in determining the etiology of pulmonary hypertension.
  • A chest X-ray. An X-ray of the chest can produce images of the heart, lungs, and chest. It might be used to look for further lung issues that could lead to pulmonary hypertension.
  • ECG or EKG stands for electrocardiogram. The heart's electrical activity is captured by this straightforward examination. Changes in the heartbeat may be visible.
  • Echocardiogram. Moving images of the heartbeat are produced using sound waves. An echocardiography reveals the heart's blood flow. This examination may be carried out to assist in the diagnosis of pulmonary hypertension or to assess the efficacy of treatment.

To better understand how exercise affects the heart, an echocardiography may occasionally be performed while using a treadmill or stationary bike. You might be required to wear a mask during this test to gauge how efficiently your heart and lungs are using oxygen and carbon dioxide.

A cardiologist performs this treatment by inserting a catheter, a small, flexible tube into a blood vessel, typically in the neck. The pulmonary artery and lower right heart chamber are both entered with care by the catheter. Following that, a physician might check the right ventricle and the major pulmonary arteries for blood pressure.

To examine the health of the lungs and pulmonary arteries, additional tests could be performed. The tests listed below may provide more details regarding the cause of pulmonary hypertension:

  • Stress tests during exercise. These examinations frequently entail using a treadmill or a stationary cycle while the heartbeat is being monitored. They can demonstrate the effects of exercise on the heart.
  • CT scan for computerized tomography. In this examination, cross-sectional images of particular body sections are produced using X-rays. A dye called contrast may be injected into a vein to enhance the visibility of the blood vessels in the images.
  • A cardiac CT scan, also known as a heart CT scan, can reveal the size of the heart and any pulmonary artery obstructions. It can aid in the diagnosis of lung conditions like COPD or pulmonary fibrosis that may cause pulmonary hypertension.
  • (MRI) Magnetic resonance imaging. This examination produces fine-grained pictures of the heart using radio waves and magnetic fields. It can reveal pulmonary artery blood flow and assess the efficiency of the right lower heart chamber.
  • Lung-function test. You blow into a unique gadget to undergo this exam. The amount of air that the lungs can contain is measured by the instrument. It demonstrates the inflow and outflow of air from the lungs.
  • Sleep study. In a sleep study, your heart rate, blood pressure, oxygen levels, and other factors are monitored while you're sleeping. Sleep apnea, which can result in pulmonary hypertension, can be identified with the test.
  • Perfusion/ventilation (V/Q) scan. A radioactive tracer (IV) is administered during this procedure. Blood flow is depicted by the tracer. Inhaling a tracer that displays airflow to the lungs is another option. If blood clots are contributing to pulmonary hypertension symptoms, a V/Q scan can reveal this.
  • Biopsy of the lungs. Occasionally, a tissue sample from the lung may be obtained to investigate a potential pulmonary hypertension etiology.
  • DNA analysis. It might be advised to conduct a gene-change screening for pulmonary hypertension. Other members of your family may need to be checked if you have these gene alterations.

Classification of Pulmonary Hypertension in Function

Depending on how the symptoms affect you and your capacity to perform daily chores, pulmonary hypertension is categorized after a diagnosis has been made.

Any of the following categories could have pulmonary hypertension:

  • Grade I. There are no symptoms during rest or exercise, despite the diagnosis of pulmonary hypertension.
  • Class II. There are no symptoms while at rest. Shortness of breath or minor chest pain might be brought on by routine tasks or activities like going to work or the grocery store. Physical activity is slightly constrained.
  • Class III. While at repose, it is comfortable, performing routine duties like getting dressed, bathing, or cooking makes it feel exhausted, causes shortness of breath, and hurts the chest. Physical exercise becomes extremely difficult to undertake.
  • Class IV. Both while you're at rest and when you're moving around, symptoms develop. Activities of all kinds result in growing discomfort.

Treatment Of Pulmonary Hypertension

Pulmonary hypertension has no known treatment option. However, there are treatments available to lessen symptoms, extend life, and prevent the condition from getting worse. Any health issues that might be causing your pulmonary hypertension may also be treated.

Finding the best treatment for pulmonary hypertension frequently takes some time. The therapies are frequently intricate. You typically require numerous health examinations.


In order to treat your symptoms and make you feel better if you have pulmonary hypertension, you may be prescribed medication. Additionally, medications may be utilized to treat or stop problems. Treatment options include:

Drugs that relax the blood vessels. These medications, which are often referred to as vasodilators, aid in widening clogged blood arteries and enhancing blood flow. You can receive it orally, intravenously, or both. A tiny pump attached to the body is used to administer some types continually.

Epoprostenol, treprostinil, iloprost, and selexipag are a few examples of vasodilators that are used to treat pulmonary hypertension.

Inhibitors of soluble guanylate cyclase (sGC). The pulmonary arteries are relaxed by this kind of medication, which also lowers lung pressure. Riociguat, an Adempas example, is one. When pregnant, avoid taking these medications.

  • Medications that widen blood vessels. The action of a chemical in blood artery walls that causes them to narrow is reversed by medications referred to as endothelin receptor antagonists. They might help with symptoms and energy levels. If you are pregnant, avoid using these medications.
  • Drugs that promote blood flow. To improve blood flow through the lungs, physicians may prescribe drugs known as phosphodiesterase 5 (PDE5) inhibitors. They include tadalafil (Adcirca, Alyq, Cialis) and sildenafil (Revatio, Viagra).
  • A high dose of calcium channel blockers. These drugs aid in calming the blood vessel walls' muscles. They consist of nifedipine (Procardia), amlodipine (Norvasc), and diltiazem (Cardizem, Tiazac, and others). Only a small percentage of persons with pulmonary hypertension experience improvement while taking calcium channel blockers, despite the fact that they can be beneficial.
  • Blood Thinners. These drugs, often known as anticoagulants, work to stop blood clots from forming. Warfarin (Jantoven), as an example. Anticoagulants slow the clotting process. The risk of bleeding may rise due to the medications. This is especially important if you're getting surgery or undergoing a procedure that involves entering the body or making an incision on the skin. Your risk should be discussed with your medical staff.
  • Digoxin, also known as Lanoxin. This drug enhances the heart's ability to pump blood more efficiently. It can assist in controlling erratic heartbeats.
  • Diuretics, water tablets. These drugs support the kidneys' function in cleansing the body of extra fluid. The labor the heart has to undertake is lessened as a result. It is also possible to take diuretics to lessen fluid retention in the lungs, legs, and abdomen
  • Treatment with oxygen. As a remedy for pulmonary hypertension, pure oxygen inhalation is occasionally advised. If you have sleep apnea or reside at a high elevation, this treatment can be recommended. Oxygen therapy may be required constantly in some pulmonary hypertension patients.

Surgery or other treatments

Surgery may be suggested if medications do not treat the symptoms of pulmonary hypertension. The following surgeries and methods may be used to treat pulmonary hypertension:

  • The Atrial Septostomy. If medications fail to control the symptoms of pulmonary hypertension, this course of treatment might be suggested. A hole is made between the upper left and right chambers of the heart during an atrial septostomy. The pressure on the right side of the heart is lessened by the opening. Arrhythmias, or irregular heartbeats, are examples of potential consequences.
  • Heart-lung or lung transplant. For certain patients with idiopathic pulmonary arterial hypertension, especially those who are younger, a lung or heart-lung transplant may be necessary. To assist in lowering the risk of rejection, medication must be taken for the rest of one's life after a transplant.

Complications Of Pulmonary Hypertension

The following are possible pulmonary hypertension side effects:

  • Failure of the heart and right-sided heart hypertrophy. This disorder, also known as cor pulmonale, causes the right lower chamber of the heart to enlarge. In order to pump blood through constricted or clogged lung arteries, the chamber must work harder than usual.

The result is a thickening of the heart walls. The capacity of the right lower heart chamber is increased by stretching. More stress is placed on the heart as a result of these alterations, and finally, the right lower heart chamber collapses.

  • Clots of blood. The danger of blood clots in the tiny arteries of the lungs rises with pulmonary hypertension.
  • Abnormal heartbeats. Arrhythmias, which are variations in the heartbeat caused by pulmonary hypertension, can be fatal.
  • Bleeding inside the lungs. Blood in the cough and life-threatening bleeding into the lungs are both possible effects of pulmonary hypertension.
  • Complications related to pregnancy. For the mother and the unborn child, pulmonary hypertension poses a serious health risk.

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