Pulmonary Hypertension Program
The Pulmonary Hypertension Program at Texas Children’s Hospital has been diagnosing and treating children with pediatric pulmonary hypertension for more than two decades. In fact, Texas Children's has one of the few pulmonary hypertension programs in the U.S. dedicated to treating children. Our experienced team has extensive training in the research, diagnosis and treatment of infants, children and teens with this rare condition, characterized by high blood pressure in the lungs.
Since its launch in 2001, Texas Children’s Pulmonary Hypertension Program has been the medical home for children, teens and young adults with pulmonary hypertension.
Texas Children’s Pulmonary Hypertension Program is dedicated to providing your child with the highest quality medical care in an open, honest and timely manner. Through our research efforts, we are focused on discovering treatment methods to improve the futures of children with pulmonary hypertension.
Pulmonary hypertension is a rare condition of high blood pressure in the lungs that makes it hard for the right side of the heart to pump blood into the blood vessels of the lungs.
At first, pulmonary hypertension symptoms are very subtle and may include breathlessness and difficulty breathing while exercising. People may change their activity level to compensate for these symptoms. Other symptoms may include chest pain, fainting and leg swelling.
If you notice your child tires easily with activity and has a history of poor growth or poor development, it may be pulmonary hypertension. In more severe cases, bluish discoloration of the lips and face may be seen with activity or your child may faint with activity. If pulmonary
hypertension progresses to heart failure, symptoms include swelling, weight gain, nausea and vomiting.
Pulmonary hypertension can occur from a variety of causes. It can occur without obvious cause or reason, can be inherited or can occur in association with another medical condition such as congenital heart disease, joint/connective tissue disease or lung disease.
Pulmonary hypertension is typically associated with poor growth, heart failure, blood clots and bleeding. When pulmonary hypertension is suspected, the screening test of choice is an echocardiogram (ultrasound of the heart). This test will give an idea of how high the pressures on the right side of the heart are. Cardiac catheterization is used to confirm the echocardiogram findings and get more detail. Both of these tests should be done in centers like Texas Children’s with pulmonary hypertension expertise so that the data is interpreted correctly.
Once the diagnosis is made by echo and/or cardiac catheterization, additional testing will be done as indicated to investigate possible causes of pulmonary hypertension. These include lung function testing, computed tomography (CT) scans of the chest, sleep studies and bloodwork.
Until the early 1990s, the only therapy for pulmonary hypertension was lung or heart-lung transplantation. We now have many drug options on the market specifically to treat pulmonary hypertension; there are other drugs that are used in conjunction with these medications but they are not specific to pulmonary hypertension. Response to therapy is monitored by checking growth, activity tolerance and 6-minute walk tests.
While the advances in medical therapy are incredible and have improved the quality and length of life for many children with pulmonary hypertension, there is no known cure for this disease. Medications only help to slow down the disease process; they don’t completely reverse it. In some children with pulmonary hypertension, lung transplantation may be an option.
At Texas Children’s, we use oral, inhaled and continuous therapies as indicated to manage pulmonary hypertension. Our team is well-versed in the various treatment options and is able to work with specialty pharmacies to ensure that your child receives the treatment they need.
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Program earns designation as a Texas Children’s Center
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