Pulmonary Hypertension Clinic

CHOC offers the only pediatric pulmonary hypertension clinic in Orange County.

Children with primary and secondary pulmonary hypertension have very specific needs. In order to make accessing the care these medically fragile children need easier and more efficient, the CHOC Pulmonary Hypertension Clinic provides multidisciplinary care that eliminates the need for multiple appointments in different facilities and brings together specialists who work together to treat the patient.

The Team Approach to Care

The Pulmonary Hypertension Clinic provides families with the opportunity to undergo tests like echocardiograms, electrocardiograms and pulmonary function tests, and meet with the pulmonologist and cardiologist, all in one clinic visit. Test results are immediate and given directly to the cardiologist and pulmonologist who team together at each appointment to come up with and maintain the very best short- and long-term treatment plans for each child.

Children referred to the Pulmonary Hypertension Clinic typically receive a pulmonary hypertension diagnosis through echocardiograms and/or cardiac catheterization prior to being referred to the clinic. Once accepted, patients are seen as needed and can also be seen by the cardiologist and pulmonologist as needed in between visits.

What is pulmonary hypertension (PPH)?

Pulmonary hypertension is a lung disorder in which the blood pressure in the pulmonary artery rises far above normal levels. There are two types of pulmonary hypertension: primary and secondary.

Primary pulmonary hypertension (PPH) is often also called “unexplained pulmonary hypertension,” because in this type of hypertension, there are no other underlying heart or lung problems causing the high blood pressure. The exact cause of PPH is unknown. Research has linked primary pulmonary hypertension to genetic or familial predisposition. Researchers believe the blood vessels are particularly sensitive to certain internal or external factors, and constrict, or narrow, when exposed to these factors, such as an immune system factor, or sensitivity to drugs or other chemicals.

Secondary pulmonary hypertension occurs as a result of the effects of other conditions, which may include diseases such as congenital heart disease or respiratory diseases at birth.

What are the symptoms of PPH?

The following are the most common symptoms for pulmonary hypertension. However, each individual may experience symptoms differently. Symptoms may include:

• Fatigue
• Difficulty in breathing (dyspnea)
• Dizziness
• Fainting spells (syncope)
• Swelling in the ankles or legs (edema)
• Bluish lips and skin (cyanosis)
• Chest pain (angina)
• Racing pulse
• Trouble getting enough air
• Palpitations, strong throbbing sensations brought on by increased heart rate.

More severe symptoms indicate a more advanced disease. In advanced stages, the patient:

• Is able to perform minimal activities
• Has symptoms even when resting
• May become bedridden if the disease becomes worse.

The symptoms of primary pulmonary hypertension may resemble other conditions or medical problems. It is important to speak with the child’s doctor if they are experiencing similar symptoms.

How is pulmonary hypertension diagnosed?

Pulmonary hypertension is rarely discovered in a routine medical examination, and in its later stages, the signs of the disease can be confused with other conditions affecting the heart and lungs.

Pulmonary hypertension is a diagnosis of exclusion. Diagnostic procedures may include:

• Electrocardiogram (EKG or ECG). A test that records the electrical activity of the heart, shows abnormal rhythms (arrhythmias or dysrhythmias), and detects heart muscle damage. Learn more about electrocardiogram.
• Echocardiogram (echo). A procedure that evaluates the structure and function of the heart by using sound waves recorded on an electronic sensor that produce a moving picture of the heart and heart valves. Learn more about echocardiogram.
• Pulmonary function tests. Diagnostic tests that help to measure the lungs’ ability to move air into and out of the lungs effectively. The tests are usually performed with special machines into which the person must breathe. Learn more about pulmonary function tests.
• Perfusion lung scan. A nuclear medicine procedure that can detect a blood clot in the artery leading to the lung. This procedure can also assess the function of the lungs.
• Cardiac catheterization. A procedure that evaluates blood flow to the heart muscle, blockage of coronary arteries, congenital heart defects, functioning of the heart valves, and other heart structures. A small catheter is advanced from a blood vessel in the groin or arm through the aorta to the heart. Learn more about cardiac catheterization.

How is pulmonary hypertension treated?

Specific treatment will be determined by your doctor based on:

• The child’s age, overall health and medical history
• Extent of the disease
• The child’s tolerance for specific medications, procedures or therapies
• Expectations for the course of the disease
• The family’s opinion or preference.

Treatment may include one or more of the following:

• Anticoagulants. These are used to decrease the tendency of the blood to clot and permit blood to flow more freely.
• Diuretics. These are used to decrease the amount of fluid in the body and reduce the amount of work the heart has to do.
• Drugs. These are used to help lower blood pressure in the lungs and improve the performance of the heart in many patients.
• Calcium channel blocking/vasodilators drugs. These are used to improve the heart’s ability to pump blood.
• Supplemental oxygen. Some patients also require supplemental oxygen delivered through nasal prongs or a mask if breathing becomes difficult.
• Lung or heart-lung transplantation.