Pulmonary Hypertension

What is pulmonary hypertension?

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Pulmonary hypertension is a type of high blood pressure. Known colloquially as PH, it occurs when the blood vessels leading to the lungs from the heart are highly pressurized. It affects the arteries in the lungs and the right side of the heart. Left untreated, the affected blood vessels can become narrowed and hardened. This puts additional strain on the heart, as it must work harder to pump the blood into the lungs. In time, this can lead to heart failure and other cardiovascular complications.

Pulmonary hypertension is a serious condition that worsens over time. If you think you may have pulmonary hypertension, schedule an appointment with your physician or cardiologist. There are certain treatments and therapies that can help improve heart function. Similarly, if you have any of the risk factors associated with pulmonary hypertension, work with your doctor to decrease your chance of developing this form of high blood pressure.

What causes pulmonary hypertension?

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Pulmonary hypertension causes can vary by person. There are five types of pulmonary hypertension, each defined by its primary cause. In all cases, the condition is caused by changes in the cells that line the affected arteries. The five types, or “groups” of PH are included below, alongside their causes.

  • Group 1: Pulmonary arterial hypertension (PAH) – This type of the condition is typically caused by a genetic mutation, congenital heart disease, and conditions that can affect connective tissue, like scleroderma and lupus. PAH may also be a result of some prescription diet drugs or methamphetamines.
  • Group 2: Pulmonary hypertension caused by left-sided heart disease – This type of pulmonary hypertension is caused by disease that affects the left-sided heart valve, such as mitral valve or aortic valve disease. Additionally, heart failure in the left ventricle can lead to this type of PH.
  • Group 3: Pulmonary hypertension with lung disease – This version of the condition is caused by lunch disease, including chronic obstructive pulmonary disease, pulmonary fibrosis, and obstructive sleep apnea. It can also be a result of long-term exposure to high altitudes in people who are already at a higher risk of developing the condition.
  • Group 4: Pulmonary hypertension caused by chronic blood clots – This one is fairly self-explanatory. It is caused by chronic blood clots in the lungs, also known as pulmonary emboli, and other clotting disorders.
  • Group 5: Pulmonary hypertension due to unknown causes – This group serves as the miscellaneous category for the condition. This form of pulmonary hypertension can arise from blood disorders, inflammatory disorders, metabolic disorders, tumors, and kidney disease. This is the hardest type to diagnose, as causes range widely.

In all cases, a separate condition leads to the development of pulmonary hypertension. If you have any of the diseases listed above, talk to your doctor about how you can mitigate your risk for developing PH.

What are common pulmonary hypertension symptoms?

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The symptoms of pulmonary hypertension are similar to most cardiovascular conditions. Common signs include:

  • Fatigue
  • Chest pain
  • Shortness of breath
  • Decreased appetite
  • Elevated resting heart rate
  • Dizziness
  • Swelling in the ankles, legs, and abdomen
  • Cyanosis, or a bluish color in the lips

Remember that symptoms of PI typically develop slowly, so it may be difficult to notice them. Symptoms get worse as the disease progresses, so speak with your doctor if you’re experiencing anything out of the ordinary.

Are there any risk factors for pulmonary hypertension?

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As with most cardiovascular conditions, there are several risk factors that will increase a person’s chance of developing pulmonary hypertension. Importantly, growing older will increase a person’s likelihood for PH. Most people diagnosed are between the ages of 30 and 60. The notable exception is that Group 1, or pulmonary arterial hypertension, is most common in younger adults.


In addition to age, there are several additional factors that can increase a person’s risk for developing PI.

  • Blood-clotting disorders
  • A genetic predisposition (a family member has the condition)
  • Being overweight
  • Use of certain drugs, like methamphetamines or cocaine
  • Living at a high altitude
  • Asbestos exposure
  • Congenital heart disease, and other genetic disorders
  • Some weight-loss drugs
  • Selective serotonin reuptake inhibitor (SSRI) use

How is pulmonary hypertension diagnosed?

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Only a doctor can diagnose pulmonary hypertension. The diagnosis process typically begins with a physical exam and medical history review, followed by a discussion of symptoms you may be experiencing. Then, the doctor will select a range of tests and diagnostic procedures to confirm the condition.

  • Confirming high blood pressure in the pulmonary arteries – The doctor will test the blood pressure in the pulmonary arteries. This is typically measured by cardiac catheterization, a procedure in which a long, thin tube is placed in the arm or upper thigh and threaded to the heart. An echocardiography can also be used to estimate pulmonary artery pressure. In most cases, the doctor will conduct the echocardiography first, and if the results suggest pulmonary hypertension, they will follow up with a cardiac catheterization.
  • Observing heart or ventricle enlargement – Doctors will likely use an array of imaging tests to confirm a diagnosis. This can include an electrocardiogram (ECG), which can reveal ventricle enlargement, chest X-rays, which provide pictures of the heart and lungs, and a computerized tomography (CT) scan, which can create images of blood vessels and tissues inside the body.
  • Determining the cause – Once a doctor provides a diagnosis, they will work to understand the cause of your pulmonary hypertension. This usually involves blood tests, biopsies, ventilation/perfusion tests, and pulmonary function tests.

How is pulmonary hypertension treated?

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There is no known treatment for pulmonary hypertension. As a result, most treatment strategies focus on mitigating symptoms and improving quality of life. This can help slow the condition’s progression. In most cases, the doctor will begin by treating the underlying condition, which will vary between people.

Some doctors will recommend pulmonary hypertension medications. This can include blood vessel dilators, which improve blood flow, and guanylate cyclase (GSC) stimulators, which relax the pulmonary arteries and lower pressure. Other medications include:

  • Endothelin receptor antagonists, which help reduce the substance that thins blood vessels
  • Diuretics, which help the kidneys remove excess fluid
  • Sildenafil and tadalafil, which can help open the blood vessels
  • Calcium channel blockers, which relax blood vessel muscles
  • Digoxin, which improves heart strength and helps control arrhythmias
  • Warfarin, a blood thinner

Your physician may also suggest oxygen therapy, especially if your PH is caused by sleep apnea or living at a high altitude.

In all cases, your doctor will recommend healthy lifestyle changes as part of your pulmonary hypertension treatment. This can improve heart function, increase a person’s ability to exercise, and help a patient work toward a healthier future.

Is there any surgery for pulmonary hypertension?

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In some severe cases, medication may not be enough to control PH. When this happens, your doctor may suggest a surgical treatment. The most common is a atrial septostomy. During this procedure, the surgeon will create a small hole between the left and right atria. This helps reduce the pressure on the right side of the heart, improving blood flow and allowing the heart to pump more efficiently.

Some doctors may suggest a balloon pulmonary angioplasty instead. In this procedure, the surgeon will use balloons to open blood vessels that have been narrowed or blocked, restoring blood flow to the lungs and reducing shortness of breath. Others will also suggest a pulmonary endarterectomy, which can remove blood clots from inside blood vessels of the lungs.

If your PH medication and lifestyle changes don’t improve your condition, talk to your doctor about surgical options. These procedures are not recommended for everybody, but only your doctor can make that determination.

What is the outlook for people with pulmonary hypertension?

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Patients who follow their treatment plans and avoid complications can expect to live relatively normal lives. You should receive routine follow-up care and monitor the condition using diagnostic and imaging tests. Importantly, make heart-healthy lifestyle changes, like diet, to help improve heart function, and engage in physical activity at your comfort level and with your doctor’s permission. Avoid high altitudes whenever possible and remember to follow treatments for underlying medical conditions.

Importantly, do not consume tobacco or nicotine, and get plenty of rest, review and get recommended vaccines, and remain as active as possible. If left untreated, pulmonary hypertension can cause major health complications, including arrhythmia, blood clots, hemoptysis (coughing up blood), heart enlargement, and heart failure. However, with routine check-ups, a strict treatment regimen, and positive lifestyle changes, people with pulmonary hypertension can lead long, fulfilling lives.