Cardiac Center

Pulmonary Hypertension

What is pulmonary hypertension?

Pulmonary hypertension (PH) is high blood pressure in the arteries of the lungs (the pulmonary arteries). Over time, the pulmonary arteries narrow, making the right side of the heart work harder as it makes the higher pressure to force blood through the narrowed arteries. In severe cases of PH, some of the small blood vessels in the lungs are damaged to the point they are lost (die).

The arteries are narrowed or lost because the muscle in the arteries contracts (vasoconstriction). Because the artery muscle cells divide and plug up the arteries (muscle cell proliferation), the vessels can become blocked with clots or the lungs can be damaged by illness, which causes the loss of air spaces and blood vessels. PH may cause the right side of your child's heart to become enlarged and eventually can cause heart failure.

Whenever possible, we start by identifying and treating the underlying problem before your child develops permanent PH. For example, if your child has a heart valve problem that is causing the PH, repairing the valve can improve the PH. Although there is no cure for PH, our treatments have become very sophisticated and we are very successful in giving children a good quality of life.


What causes pulmonary hypertension?

Your child may have developed PH for many reasons; sometimes children will have more than one cause. PH is classified into five major groups:

Group 1

True pulmonary arteriolar hypertension as a result of some, but not all, forms of congenital heart disease, connective tissue disease or sickle cell disease.

Problems that can lead to Group 1 PH include: patent ductus arteriosus (PDA), ventricular septal defects (VSDs) and atrial septal defects (ASDs). These defects can cause damage to the blood vessels by allowing too much blood to flow through your child's lungs (the condition is also called Eisenmenger syndrome). Transposition of the great arteries is also associated with PH in about 5 percent of children with PH. PH sometimes has a genetic cause and can be seen in family members. Some of these genetic causes can be diagnosed by specialized genetic testing.

Group 2

PH that is caused by failure of the left side of the heart, in other words, the pressure backs up into the lungs.

Heart problems that can lead to Group 2 PH include:

Group 3

PH that is caused by any disease that primarily affects the air spaces in the lungs. The Cardiac Center’s Pulmonary Hypertension Program treats many infants and children who have Group 3 PH as a result of chronic lung disease of prematurity (Bronchopulmonary Dysplasia (BPD)) or lung hypoplasia (lungs too small) after repair of congenital diaphragmatic hernia (CDH) or other lung lesions.

Group 4

PH that is caused by blood clots or thrombosis in the blood vessels in the lung.

Group 5

A combination of many rare diseases that rarely also cause PH.


How is pulmonary hypertension diagnosed?

Although PH is rare in children, it is being diagnosed more frequently. As of July 2013, our program has been actively following more than 650 children and adolescents with pulmonary hypertension. PH may show up at any age and often symptoms are thought to be from other more common childhood diseases. Symptoms include fatigue (usually the first symptom to develop); difficulty breathing; dizziness; fainting, especially with activity; swelling and/or discoloration in the lower legs or ankles; chest pain; or a bluish discoloration of the lips.

Our evaluation starts at the first phone call or Hospital visit, when we evaluate the history and symptoms to determine how urgently your child needs to be seen or treated. During your child's evaluation for PH:

If heart disease is found during the testing, we may be able to treat it or even repair it with surgery. If lung disease is identified as a cause of the PH, we work closely with our world-renowned colleagues in the Division of Pulmonary Medicine as well as other specialties, such as the Pulmonary Hypoplasia Program and the Neonatal and Infant Chronic Lung Disease Program.


How is pulmonary hypertension treated?

Treatment of pulmonary hypertension may include medications that relax the blood vessels in your child's lungs; anticoagulants that reduce clotting and help blood flow; diuretics to reduce the amount of fluid in your child's body; medications so that the heart doesn't have to work as hard; and oxygen to relax the blood vessels in the lung.

There are currently no FDA-approved therapies for pediatric pulmonary hypertension; however, our program has more than 20 years of experience with the careful use of all therapies that are FDA-approved for adult PH. We are also working with new medications that stop the muscle cells from plugging up the vessels of the lung. All therapies, including newer and more experimental treatments for pediatric PH, are available in our dedicated clinic.

Some children with pulmonary hypertension eventually require lung or heart-lung transplantation, but we first use aggressive medical treatments that may improve your child's health and quality of life.

Because PH is a rare and complex disease and can be difficult to diagnose, your child's cardiologist should have special expertise in the disease. At The Children's Hospital of Philadelphia’s Cardiac Center, your child is treated by a team of world-renowned experts. Our team includes pediatric cardiologists, cardiac anesthesiologists, pulmonologists, nurse practitioners, nurse coordinators, psychologists and exercise physiologists with expertise in pediatric pulmonary hypertension.

In addition to providing the finest clinical care, we are actively engaged in research. We are a founding institution of the Pediatric Pulmonary Hypertension Network and we lecture on pediatric PH both nationally and internationally. Brian Hanna, MDCM, PhD, FAAP, FACC, is the medical director of a sleep-away summer camp for children with complex cardiac and lung issues, including PH.


Schedule an appointment for pulmonary hypertension evaluation

If you have a child or patient with suspected pulmonary hypertension, please call our office at 215-590-5248 for an immediate consultation or evaluation. For an emergent consultation, please call 215-590-1000 and ask for the cardiologist on call for pulmonary hypertension.

The Cardiac Center offers clinic appointments daily for children with PH. We care for our patients throughout the inpatient and outpatient facility and have PH-dedicated staff on call 24 hours a day. Since we treat more than 20 inpatients with pulmonary hypertension on a given day, PH is understood by every member of the CHOP family — from the Emergency and Transport Departments to the chefs in the kitchen.

To learn more about the disease and connect with other families and professionals, visit the Pulmonary Hypertension Association website, which has helpful resources and information for both families and clinicians.


Reviewed by: Brian Hanna, MDCM, PhD, FAAP, FACC
Date: September 2013

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