When small arteries in the lungs are inflamed, they can become damaged, narrowed or blocked, making it harder for blood to flow normally. This pressure—known as pulmonary hypertension— builds up and weakens the lungs and right side of the heart. Over time, the artery walls can stiffen or tighten and blood clots can form.

With some types of pulmonary hypertension, symptoms can go unnoticed. For other types, pulmonary hypertension symptoms can be severe and can be life-threatening. All types of pulmonary hypertension worsen over time. While there is no cure, lifestyle changes, treatment and possibly surgery, can help improve the symptoms and quality of life.

Baptist Health is known for advanced, superior care for patients with heart disease and the diagnosis, management and treatment of pulmonary hypertension. You will appreciate timely appointments and respectful attention to your concerns, all in a positive and friendly atmosphere. Here, you have access to the region’s most comprehensive, multidisciplinary team of specialists and innovative therapies, including many available only through specialized clinical trials. In every way, we work to demonstrate the utmost in excellent care to those who trust us with their health.

Signs and Symptoms

Pulmonary hypertension symptoms can vary. Some people experience no symptoms for months or years. For others, symptoms range from mild to severe. Over time, symptoms worsen for all types of pulmonary hypertension, including:

• Bluish tint of the nails, lips and skin
• Chest pain
• Decreased appetite
• Dizziness, especially during activity
• Enlarged jugular vein in the neck
• Fatigue
• Fluid retention causing swelling in the ankles, legs, feet and/or abdomen
• Rapid or irregular heartbeat
• Shortness of breath that worsens progressively when exercising, and breathing when resting

Diagnosis

Pulmonary hypertension is rarely diagnosed early. To determine a diagnosis of pulmonary hypertension, we use advanced technology to effectively diagnose, inform treatment and carefully monitor the condition.

Diagnostic procedures and technologies can include:

Blood tests: Tests check for certain substance that can indicate pulmonary hypertension to rule out other conditions.

Right heart catheterization: A long, thin flexible tube is threaded through a blood vessel in the groin or neck and to the heart. Contrast material is injected through the tube and a type of X-ray and still and moving pictures are taken to show how your heart functions.

CT scan: X-rays and computers are used to create images of the chest to look for blood clots and lung conditions.

Chest X-ray: A common imaging test of the chest, heart and lungs.

Echocardiogram: This ultrasound exam uses soundwaves to take moving pictures of the heart’s chambers and valves. At times, riding a bike or walking on a treadmill is necessary to measure how well the heart and lungs work when stressed.

Magnetic resonance imaging (MRI): A large magnet, radio waves and a computer are used to produce pictures of the heart and blood vessels.

Pulmonary function test: This test measures how much air can be held in the lungs and the airflow in and out of the lungs by blowing into a spirometer.

Ventilation/perfusion (V/Q) scan: For the ventilation scan, a small amount of radioisotope gas is inhaled. For the perfusion scan, radioisotopes are injected into a vein in the arm. The low-level radioisotopes can be traced using a special scanner, and images can be created of air and blood flow patterns in the lungs.

Causes

Causes that lead to the development of pulmonary hypertension can include:

• Cocaine use
• Diet drugs
• High blood pressure  
• Obesity
• Sleep apnea

Risk Factors

Pulmonary hypertension risk factors can include:

Age: Idiopathic (unknown cause) pulmonary hypertension is more common in young adults.

Blood clots in the lungs: These clots can be chronic and block blood flow in the arteries of the lung.

Chronic diseases: Liver diseases, including cirrhosis; rheumatic disorders, including lupus; lung conditions, including emphysema and chronic obstructive pulmonary disease (COPD); sickle cell disease; and HIV.

Heart diseases: Mitral valve disease, left-heart failure, aortic valve disease.

Congenital heart defects: Structural heart defects present at birth.

Family history: In some cases, a gene can be inherited that leads to pulmonary hypertension.

Long-term exposure to high altitudes: Lower levels of oxygen in this environment can stress the heart and lungs.

Sleep apnea: This sleep disorder deprives the body of normal levels of oxygen.

Prevention

While you cannot prevent all types of pulmonary hypertension, you can take steps to lower your risks of complications by following these healthy lifestyle steps:

Be active: Moderate exercise helps circulation and decreases stress on your heart muscle.

Eat a healthy diet: Limit salt, sugar, saturated fat and cholesterol; and eat plenty of fruit, vegetables, whole grains and low-fat dairy products.

Get regular checkups: And, if you experience new or changing symptoms or side effects from medications, see your physician.

Reduce stress: Stress can contribute to a fast or irregular heartbeat.

Stop smoking: Smoking damages blood vessels, raises blood pressure, reduces the amount of oxygen in the blood and makes the heart beat faster.

Take your medications as prescribed:  If you’ve been prescribed a medication for pulmonary hypertension or a causative condition, be sure to take it as prescribed.

Prognosis

A prognosis depends on the type and severity of the pulmonary hypertension and any underlying causes. Pulmonary hypertension cannot be cured. But symptoms can be managed with appropriate treatment, which may be involved and require extensive follow-up care. At times, treatments lose effectiveness and must be replaced with new ones.

Treatment and Recovery

Pulmonary hypertension treatment can include the following medications:   

• Blood thinners: To reduce of the possibility of blood clots forming in the lungs or heart.
• Calcium-channel Blockers: To relax the walls of blood vessels.
• Diuretics: To help eliminate extra fluid and reduce the amount of work for your heart.
• Oxygen therapy: To help with breathing for those who live at high altitudes or who have sleep apnea.
• Other drugs: To lower blood pressure in the lungs and help the heart beat stronger to pump more blood.
• Vasodilators: To open narrowed blood vessels. The drug can be injected through a small tube connected to a small pump worn on the belt or shoulder. Or, the medication can be inhaled using a nebulizer.

If medications are not effective, your physician may recommend the following therapy or surgeries:

• Atrial septostomy: Open-heart surgery to relieve pressure on the right side of the heart by creating a small opening between the upper right and left chambers.
• Lung or heart transplant surgery: During a transplant surgery, the unhealthy organ is removed and replaced with a healthy one from a deceased donor.

Recovery After Surgery

After a lung or heart transplant, recovery varies by patient, but staying in the hospital one to two weeks is normal and full recovery can take up to three months. Rehabilitation can begin in the hospital; rehabilitation team members provide counseling, and education about medications, a healthy diet, smoking cessation, possible side effects of surgery and medications, and an exercise program to regain strength.

The following side effects of surgery can include:

• Itchiness from incisions healing
• Tightened shoulders and upper back muscles
• Tiredness, moodiness or depression
• Loss of sleep or appetite
• Pain at the site of the chest bone incision

Your physician will tell you when you can be physically active again. Most people can resume sexual activity within four weeks and driving after three to eight weeks. Many return to work within six weeks, unless they have a physically demanding job. Do not exercise or lift heavy objects without a physician’s approval.

Follow-up Care After Surgery

A transplanted heart is a foreign object that the immune system tries to reject, so it is important to take medications as prescribed to prevent rejection. Establish a daily routine for taking all medications at the same time each day, and for refills. A transplant center team will monitor the new organ to watch for signs of rejection. It is important to call a physician immediately if any symptoms of rejection occur:

• Fatigue
• Fever
• Shortness of breath
• Urinating less can indicate a problem with the kidneys
• Weight gain caused by retaining fluid

The medication prescribed to fight rejection lowers the immune system’s ability to fight infection. Guard against infections by avoiding crowds and contact with animals. Call a physician if any of these signs of infection occur:

• Cough
• Drainage at the site of an incision
• Phlegm with color
• Redness or tenderness
• Shortness of breath
• Swelling

Recovery can be emotionally overwhelming for some, causing anxiety and depression. Talk with a physician about medication to stabilize moods. Emotional support is available from rehab counselors and support groups. It is also important to seek the support of family and friends.

Complications

The most common complications of pulmonary hypertension are:

Right-sided heart enlargement and heart failure: As the heart has to pump harder, the right ventricle becomes enlarged and weakened. Over time, this condition can lead to heart failure, which can be life-threatening.

Blood clots: Having pulmonary hypertension makes it more likely to develop blood clots in the lungs.