Understanding Primary Pulmonary Hypertension: Symptoms, Causes, Treatment & Prevention

Tampa General Hospital’s esteemed Heart & Vascular Institute diagnoses and treats primary pulmonary hypertension (PPH), a relatively rare condition. Pulmonary hypertension causes high blood pressure within the pulmonary arteries. These vessels carry deoxygenated blood from the right side of the heart to the lungs, where the blood then releases carbon dioxide and collects oxygen before flowing back to the heart and throughout the rest of the body. Primary pulmonary hypertension, more specifically, occurs when the blood vessels within the lungs narrow for no known cause, causing blood pressure within the pulmonary arteries to increase far beyond what is considered normal.

Also referred to as idiopathic pulmonary arterial hypertension (IPAH), primary pulmonary hypertension is a serious, chronic condition that requires medical attention from highly skilled and experienced pulmonary experts like the ones at Tampa General Hospital. If primary pulmonary hypertension isn’t properly treated, it can lead to heart failure, which can in turn result in death.

What Are the Causes & Risk Factors of Primary Pulmonary Hypertension?

Researchers are still working to determine exactly what causes primary pulmonary hypertension. Research also suggests that primary pulmonary hypertension could have a genetic component, meaning that a certain gene defect increasing blood vessel sensitivity could cause the condition to run in families. Additionally, dysfunction with the endothelial cells that line the pulmonary arteries is believed to play a role in the development and progression of PPH.

What Are the Symptoms & Warning Signs of Primary Pulmonary Hypertension?

The symptoms of primary pulmonary hypertension vary from one person to another. With that said, if you have primary pulmonary hypertension, you may experience:

• Chest pain (angina)
• A racing pulse
• Heart palpitations (arrhythmia)
• Difficulty breathing or shortness of breath (especially during activity)
• Dry coughing
• Blue-tinged lips and skin
• Swollen legs and ankles
• Dizziness
• Fainting (syncope)
• Fatigue

These symptoms often develop gradually over the course of many years, which means that many individuals with primary pulmonary hypertension don’t seek treatment until the condition has already progressed to a more advanced stage. Plus, many of the symptoms of primary pulmonary hypertension are also associated with other conditions affecting the heart and lungs. For these reasons, it’s important to reach out to a physician as soon as you begin experiencing any of the symptoms listed above.

Common Symptoms of Advanced Primary Pulmonary Hypertension

The symptoms of primary pulmonary hypertension tend to worsen as the condition progresses. Once the disease reaches an advanced stage, symptoms may occur even when someone is resting. Symptoms may also become so severe that they cause someone to become bedridden.

Diagnosis & Tests for Primary Pulmonary Hypertension

Primary pulmonary hypertension typically begins with a discussion of your symptoms and medical history, along with a thorough physical examination. If your doctor suspects that you might have primary pulmonary hypertension, they’ll likely order one or more of the following tests to confirm the diagnosis:

• Blood tests – Depending on the type of blood work you’re having completed, a medical provider may use it to measure oxygen levels in your blood or monitor any strain on your heart.
• Cardiac catheterization – This test enables your physician to measure the amount of pressure within your pulmonary arteries. Right heart catheterization (RHC) is used specifically for diagnosing IPAH.
• Chest X-ray – This type of imaging allows your doctor to view your heart, lungs and other internal tissues within your chest.
• Computed tomography (CT) scan – This imaging method creates detailed pictures of the inside of your body.
• Echocardiogram – This test produces sound waves to create a moving image of your heart.
• Electrocardiogram (ECG or EKG) – The heart generates electrical impulses that help control how quickly it beats, and this test records the strength and timing of that electrical activity.
• Lung perfusion scan – This nuclear radiology test shows how both air and blood flow through your lungs.
• Pulmonary function tests (PFTs) – These tests measure how well your lungs work. For example, a spirometry test measures how much air you’re able to exhale, as well as the speed at which you’re able to do it.

How Do You Treat Primary Pulmonary Hypertension?

Researchers are working to identify a cure for primary pulmonary hypertension. As a result, treatment is often focused on managing symptoms. If your physician diagnoses you with primary pulmonary hypertension, they’ll develop a care plan that’s customized to your specific needs, possibly including some combination of medication, oxygen therapy and surgery.

Medication

Many individuals with primary pulmonary hypertension are able to manage their symptoms by taking one or more of the following medications:

• Anticoagulants – Commonly referred to as blood thinners, anticoagulants are specially designed to help prevent blood clots from forming, thereby helping blood flow more easily through the body.
• Calcium channel blockers – Calcium can cause the heart and arteries to squeeze more tightly. This medication prevents calcium from entering the heart and artery cells, which helps relax the heart muscle and widen the arteries. This, in turn, reduces the strain on the heart, promotes easier blood flow and lowers blood pressure.
• Diuretics – Also known as water pills, diuretics stimulate the kidneys to produce more urine, allowing the body to excrete more fluids and salt. Reducing the amount of fluid in the body can ease swelling and decrease the amount of strain on the heart.
• Vasodilators – These medications help relax the walls of blood vessels, which allows blood to flow more easily through those passageways. By doing so, they help reduce blood pressure within the lungs.

Oxygen Therapy

If primary pulmonary hypertension has made it more difficult for you to breathe, you may be a candidate for oxygen therapy. Also referred to as supplemental oxygen, oxygen therapy is a treatment method that involves administering extra oxygen, often through either nasal prongs or a face mask. If your doctor determines that you could benefit from oxygen therapy, they’ll recommend the device that’s best suited to your specific needs and walk you through how to use that equipment.

Surgery

Especially severe, end-stage cases of primary pulmonary hypertension may require either a lung transplant or a heart-lung transplant. This type of surgery is typically reserved for individuals who have attempted all other treatment options available to them but failed to find sufficient relief from their symptoms.

What Are the Complications & Long-Term Effects of Primary Pulmonary Hypertension?

Failing to properly treat primary pulmonary hypertension can lead to heart failure, which is why it’s so important to consult with a medical provider if you’re experiencing any of the symptoms described above. Heart failure is a potentially life-threatening condition that occurs when the heart doesn’t pump blood as well as it should, often causing blood to back up in the veins and lungs. Because the heart can’t supply enough blood for critical bodily functions, heart failure can produce symptoms throughout the body, including:

• A rapid or irregular heartbeat
• Shortness of breath (especially when exercising or lying down)
• Wheezing
• Coughing that persists or produces bloody mucus
• Swollen abdomen, legs, ankles and feet
• Rapid weight gain due to fluid buildup
• Nausea
• Reduced appetite
• Fatigue
• Weakness
• Difficulty concentrating
• Decreased alertness

How Do You Prevent & Reduce Your Risk of Primary Pulmonary Hypertension?

Unfortunately, because there’s currently no known cause of primary pulmonary hypertension, there’s little you can do to outright reduce your risk of developing this condition. With that said, it’s important to live a healthy lifestyle and monitor yourself for the symptoms listed above. Because primary pulmonary hypertension is a progressive condition, an early diagnosis can help ensure that you have the greatest number of treatment options available to you and allow for a better outcome.

Living With Primary Pulmonary Hypertension

Depending on the severity of your symptoms, primary pulmonary hypertension can take a considerable toll on your quality of life, possibly preventing you from engaging in the activities you once enjoyed. To minimize your symptoms and remain as healthy as possible, you should:

• Avoid activities that could cause chest pain, dizziness or other dangerous symptoms
• Keep up with recommended vaccinations (e.g., annual flu shots)
• Quit smoking (if applicable)

While exercising is typically an excellent way to stay healthy, you should consult with your physician before beginning a new exercise program to ensure that it’s safe. You should also consult with your doctor before taking any new medications (including both prescription and over-the-counter medicines), flying, traveling to locations with a high altitude or getting pregnant.

Primary Pulmonary Hypertension Frequently Asked Questions (FAQs)

Below are answers to some of the questions we commonly receive about primary pulmonary hypertension.

Do I Have Primary Pulmonary Hypertension or Am I Just Out of Shape?

Many people delay seeking treatment for IPAH because they believe their symptoms are simply a result of them being out of shape. Indeed, many of the symptoms of primary pulmonary hypertension are common among individuals who are unfit, including shortness of breath during exercise, an increased heart rate and fatigue. However, shortness of breath with minimal exertion, regardless of fitness level, is a key indicator of a problem.

Because primary pulmonary hypertension can lead to such serious complications, it’s important to consult with a physician anytime you’re experiencing the symptoms of this condition. This is especially true if your symptoms worsen over time, occur when resting or include chest pain, heart palpitations, blue-tinged lips or skin, leg and ankle swelling, dizziness or fainting. Even if it turns out that you’re just out of shape and don’t have primary pulmonary hypertension, a doctor can recommend steps you can take to become healthier.

What’s the Difference Between Primary & Secondary Pulmonary Hypertension?

Pulmonary hypertension—a condition that causes high blood pressure in the pulmonary arteries—can be classified into two main types: primary pulmonary hypertension and secondary pulmonary hypertension. Primary pulmonary hypertension has no known cause; this is why it’s commonly referred to as idiopathic pulmonary arterial hypertension. Secondary pulmonary hypertension, on the other hand, has known causes, some of the most common being underlying heart and lung diseases.

Does Primary Pulmonary Hypertension Go Away?

Primary pulmonary hypertension doesn’t have any known cure, so the condition will not go away entirely. However, there’s still hope for individuals who have been diagnosed with primary pulmonary hypertension. Many treatments have been identified that can help manage the symptoms of this condition, including medication, oxygen therapy and certain lifestyle changes. People with especially severe cases of primary pulmonary hypertension may also be able to find relief by undergoing a lung or heart-lung transplant. By monitoring yourself for symptoms and consulting with a physician about any suspected issues, you can help improve your prognosis and ensure that you have access to the greatest number of potential treatments.

Is Drinking Water Good for Primary Pulmonary Hypertension?

While staying hydrated certainly offers many benefits, individuals with primary pulmonary hypertension need to carefully manage their fluid intake. When your body contains excess fluid, your heart has to work harder to circulate those fluids throughout your body, which can aggravate the symptoms of primary pulmonary hypertension. This is why many people with this condition take diuretics.

Your doctor can provide you with personalized advice about how much fluid to consume each day. It’s important to remember that fluids not only come from beverages, but also from certain foods. So, be sure to also monitor how many fluid-rich foods you consume (e.g., soup, ice cream and fruits and vegetables with a high water content).

The TGH Difference for Primary Pulmonary Hypertension

When experiencing the symptoms of primary pulmonary hypertension—including chest pain, a racing pulse, heart palpitations, difficulty breathing, shortness of breath, dry coughing, blue-tinged lips and skin, swollen legs and ankles, dizziness, fainting and fatigue—it’s important to promptly consult with a medical provider who’s experienced in treating this condition. Delaying treatment could increase your risk of experiencing potentially life-threatening complications such as heart failure.

The skilled teams at Tampa General Hospital’s Heart & Vascular Institute and the Pulmonary Vascular Diseases Program expertly diagnose and treat primary pulmonary hypertension. Not only did U.S. News & World Report name us One of the Nation's Best Hospitals for Heart & Vascular Care and for Pulmonology and Lung Surgery, but we’ve also been recognized by Becker’s Hospital Review (which included us on its list of 100 Hospitals and Health Systems with Great Heart Programs) and Newsweek (which ranked us as one of America’s Best Specialized Hospitals for Cardiac Care in 2024).

At Tampa General Hospital, we treat a range of conditions affecting the cardiac and vascular systems, so you can feel confident entrusting us with your care. Our multidisciplinary team of board-certified pulmonologists, cardiac and vascular surgeons, general cardiologists, interventional cardiologists, heart failure specialists, electrophysiologists, radiologists and specialized pulmonary, cardiac and vascular care nurses and technologists works together to provide patients with a highly individualized care experience. Plus, our partnership with the University of South Florida positions our teams to be at the forefront of groundbreaking research and advanced clinical trials.

Contact Tampa General Hospital’s Heart & Vascular Institute to learn more about primary pulmonary hypertension diagnosis and treatment. Call (813) 844-3900 or email heart-vascular@tgh.org.