About Pulmonary Hypertension

What is pulmonary hypertension?

Pulmonary hypertension is a rare disease caused by high blood pressure in the arteries of your lungs, the pulmonary arteries. The heart pumps blood through these arteries to the lungs, where it becomes oxygenated and is returned back to the heart. When pressure in the pulmonary artery is high, the right side of the heart has to work harder. This extra effort eventually damages both the lungs and the heart. Left untreated, pulmonary hypertension can result in right heart failure.

Unlike blood pressure in the rest of the body's arteries, pulmonary artery blood pressure cannot be measured with a cuff, and pulmonary hypertension is therefore challenging to diagnose and monitor.

There are many types of pulmonary hypertension, which have been grouped by the World Health Organization into five categories based on the underlying cause.

Pulmonary hypertension can be inherited or caused by a genetic defect or as part of a congenital syndrome. It can be caused by exposure to certain toxins, such as methamphetamines. Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare type of pulmonary hypertension caused by blood clots in the arteries of your lungs. Idiopathic pulmonary hypertension refers to pulmonary hypertension that has no known cause. 

Other types of pulmonary hypertension are the result of health conditions including:

• Congenital heart disease
• Pulmonary vein stenosis
• Chronic lung diseases/bronchopulmonary dysplasia
• Congenital diaphragmatic hernia, omphalocele, and other developmental lung diseases
• Scleroderma, lupus, and other connective tissue diseases
• HIV disease
• HHT (hereditary hemorrhagic telangiectasia)
• Liver and kidney diseases
• Sickle cell disease
• Chronic lung disease
• Advanced lung failure

In children, pulmonary hypertension is most commonly caused by congenital heart disease or lung disease. Children of all ages can be affected by pulmonary hypertension. Persistent pulmonary hypertension of the newborn is caused by a failure of the circulation to transition after birth. 

Pulmonary Hypertension in Newborns

Symptoms of Pulmonary Hypertension

Symptoms of pulmonary hypertension vary and are often associated with the condition that is causing the disease. Symptoms can include:

• Difficulty breathing when exercising or at rest
• Chest pain
• Fainting
• Swelling of the legs

Most of these symptoms can be associated with other conditions, so it is important that you or your child is seen by a specialist if pulmonary hypertension is suspected.

In children, diagnosis can take time because symptoms are commonly associated with less severe issues. 

Diagnosis of Pulmonary Hypertension

Pulmonary hypertension is difficult to diagnose and requires a team of experts to make a complete and accurate assessment. Patients who are referred to our center often have already completed a series of preliminary tests—including blood tests, chest X-rays, echocardiogram, and electrocardiogram—which have led to a suspicion of pulmonary hypertension.

Our team uses an extensive set of tools, paired with the expertise of cardiologists, geneticists, pulmonologists, and others to complete an accurate diagnosis. We perform more than 150 cardiac catheterizations—the gold standard for pulmonary hypertension diagnosis—each year. 

During your first appointment with a physician at our center, we will review your medical history and schedule one or more of the following tests:

• Imaging tests: Imaging tests, such as X-ray, echocardiogram, and MRI help us to see the structure and function of the heart and lungs.

• Laboratory Testing: Certain blood tests help us diagnose pulmonary hypertension.

• Exercise testing: You may be asked to perform a six-minute walk or other form of exercise to determine your tolerance for exercise.
• Pulmonary function tests: These tests measure your lung’s ability to hold air and exchange oxygen, as well as how much air moves in and out of your lungs. These tests can help identify the cause of your pulmonary hypertension.
• Genetic testing: Some key genetic mutations that are associated with pulmonary hypertension have been identified. Genetic testing can determine whether yours is an inherited disease and/or whether family members should also be tested for a mutation so that they can understand their risk of developing the disease.
• Nuclear imaging: A nuclear scan may be scheduled if blood clots in your arteries are suspected. Nuclear scans involve small doses of radioactive material that is inhaled or injected into the lungs.
• Cardiac catheterization: This test, commonly referred to as "right heart catheterization,” will enable a definitive diagnosis of pulmonary hypertension. A long, thin tube called a catheter is guided to the right side of the heart and lungs through a tiny incision in the groin or neck. Physicians can then directly measure the pressure inside the pulmonary arteries, information that is critical to an accurate diagnosis.

Once we have determined the type and cause of your pulmonary hypertension, we will work closely with you and your family to help you understand your diagnosis and your treatment options. 

Pulmonary Hypertension Treatment

Classification by Severity

When you have been diagnosed with pulmonary hypertension, your disease will most likely be classified according to its severity. All forms of pulmonary hypertension need to be treated, but the type of treatment will depend on the severity. 

• Class I: You or your child has no symptoms of pulmonary hypertension, either when exercising or at rest.
• Class II: You or your child has no symptoms at rest but you may experience shortness of breath, fatigue, or other symptoms during normal activity. 
• Class III: You or your child has no symptoms at rest but activity is very limited by symptoms such as shortness of breath, fatigue, and chest pain.
• Class IV: You or your child has symptoms both at rest and during activity. 

Complications of Pulmonary Hypertension

Pulmonary hypertension is a serious disease that can lead to life-threatening complications, including:

• Right heart failure: Your heart's right ventricle has to work harder to pump blood through narrowed or blocked pulmonary arteries and into the lungs. Eventually, the strain on the right ventricle causes a thickening of its walls and enlarging of the heart chamber, which ultimately leads to heart failure. 
• Arrhythmia: Irregular heartbeats, called arrhythmias, are complications of pulmonary hypertension that can lead to palpitations, dizziness, or fainting. 
• Blood clots: People with pulmonary hypertension are more likely to develop blood clots in their lungs’ arteries. This is especially dangerous when the arteries are narrowed or blocked. 
• Bleeding: Bleeding in the lungs and coughing up blood are serious and potentially fatal complications.