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Pulmonary Hypertension Clinic

CHOC Children’s 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 Children’s 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.

Pulmonary Hypertension

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:

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

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:

How is pulmonary hypertension treated?

Specific treatment will be determined by your doctor based on:

Treatment may include one or more of the following:

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UC Irvine

CHOC Children's is affiliated with the UC Irvine School of Medicine