Testing and diagnosis of pulmonary hypertension

Our involvement 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:

• We will perform a thorough physical exam and may order testing. If you have reports and images from previous studies, we will ask to review them and we may perform our own, including a very complete echocardiogram (ultrasound of the heart).
• We will determine if exercise testing is possible, safe, and appropriate, even if it is just a short monitored hall walk.
• We may order blood tests. Simple blood tests can confirm our assessment of the severity of heart failure and can be extremely important in determining the cause and planning treatment.
• We may perform a cardiac catheterization, which is the gold standard to provide a definitive diagnosis of PH, to determine the severity of PH, and guide our treatment. Because cardiac catheterization is an invasive procedure we require the assistance of our cardiac anesthesiologists and often a night’s stay in our Evelyn and Daniel M. Tabas Cardiac Intensive Care Unit. Because we perform hundreds of catheterizations in children with PH, we can identify and minimize the risks of this procedure. During the procedure, a catheter is threaded through blood vessels into the right side of the heart to the pulmonary artery. With this catheter, we measure the pressures in the pulmonary vessels and the blood flow into the lungs to help us determine the severity of the condition and confirm the diagnosis. We may give your child medications during the catheterization so that we can see how treatment will affect the pressure in the pulmonary artery. Catheterization can help us predict disease progression.

Because PH is a rare and complex disease and is difficult to diagnose, your child's treatment team should have special expertise in the disease. At Children's Hospital of Philadelphia, we provide in-hospital treatment to more than 20 children with PH every day, so PH is understood by every member of your CHOP team, from the emergency room to the intensive care.

Treatment for pulmonary hypertension in children

Whenever possible, we start by identifying and treating the underlying problem before your child develops permanent and life-threatening pulmonary hypertension. For example, if your child has a heart valve problem that is causing the PH, repairing the valve can improve the PH. If heart disease is found during testing, we may be able to treat it or even repair the problem 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. With over 20 years of experience and hundreds of infants with PH and lung disease, our protocols have been extremely successful; however, the earlier we can start treatment, the better our outcomes are going to be.

Treatment of pulmonary hypertension may include:

• Therapies to eliminate the cause of the vessel damage
• Oxygen to relax the blood vessels in the lung
• Medications that relax and promote growth of the blood vessels in the lungs
• Anticoagulants that reduce clotting and help blood flow
• Diuretics to reduce the heart failure and the amount of fluid in the body
• Medications so that the heart doesn't have to work as hard

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 PH clinic.

Outlook for pulmonary hypertension

Although there is no cure for PH, our treatments have become very sophisticated. Also, the earlier the condition is diagnosed and treated, the better we're able to control it. At CHOP, we have been very successful in providing children with a good quality of life.

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. Our transplant team has an extensive experience with children with PH, and CHOP’s Pulmonary Hypertension Program is well integrated into both pre-and post-operative transplantation care.

In addition to providing the finest clinical care, we are actively engaged in research and advocacy to advance the understanding and treatment of pediatric PH. We are a founding institution of the Pediatric Pulmonary Hypertension Network, we participate in multi-centered research trials, and we lecture on pediatric PH both nationally and internationally. We pride ourselves in the partnership with families and children that ensures the best outcomes for all children with PH.