Pulmonary hypertension (PH) is high blood pressure in the arteries of the lungs (the pulmonary arteries). Over time, this pressure causes the pulmonary arteries to narrow, making the right side of the heart work harder as it forces blood through the narrowed arteries.
The arteries are narrowed because the muscle in the arteries contracts (vasoconstriction) and because the muscle cells divide and plug up the arteries (muscle cell proliferation). PH may cause your child's heart to become enlarged and eventually cause heart failure.
Whenever possible, we repair 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 usually stops 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.
Your child may have developed PH as a result of severe congenital heart disease, lung disease, connective tissue disease or sickle cell disease; this is called secondary pulmonary hypertension. Heart problems that can lead to secondary pulmonary hypertension, include:
PH sometimes has a genetic cause and is seen in family members. Several of these genetic causes can be diagnosed by DNA testing.
Although PH is rare in children, it is being diagnosed more frequently. PH may show up at any age. Symptoms include fatigue (usually the first symptom to develop); difficulty in breathing; dizziness; fainting; swelling in the legs or ankles; chest pain; and a bluish discoloration of the lips.
Our evaluation includes performing cardiac catheterization to determine the severity of the condition and guide our treatment. In this procedure, we thread a catheter through blood vessels into the right ventricle and pulmonary artery. The catheter measures the pressure in the pulmonary artery so that we may confirm or rule out PH; determine the severity of the condition if it is PH; and predict the disease progression. We may also give your child several medications during the catheterization so that we can see how each medication affects the pressure in the pulmonary artery.
If congenital heart disease is found during the testing, we may be able to repair it with surgery.
Treatment 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 so that the heart doesn't have to work as hard; and oxygen.
We are also working with new medications that stop the muscle cells from plugging up the vessels of the lung. All FDA-approved therapies as well as newer and more experimental treatments for pediatric PH are available in our dedicated clinic.
If your child has permanent pulmonary hypertension, we will use a combination of oxygen, medications and cardiac exercise rehabilitation to treat the disease. Some children with PH eventually require lung or heart-lung transplantation, but we first use aggressive medical treatment options 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 doctor should have special expertise in the disease. At the The Children's Hospital of Philadelphia Cardiac Center, your child is treated by a team of world experts. In addition to providing among the finest clinical care, we are engaged in research and publication of our results.
Our team consists of pediatric cardiologists with expertise in pediatric PH, nurse practitioners, nurse coordinators, psychologists, exercise physiologists and cardiac anesthesiologists with specialized PH experience.
If you have a child or patient with suspected pulmonary hypertension, please call our office at 215-590-5248 for an evaluation or consultation.
To learn more about the disease and connect with other families and professionals, visit the Web site of the Pulmonary Hypertension Association, which has helpful resources and information for both families and clinicians.
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