Pulmonary Hypertension Clinic
The Pulmonary Hypertension (PAH) Clinic at Children's Hospital & Medical Center has been treating pediatric PAH patients for the past decade and is the only pediatric clinic of its kind in the state. Once your child has been diagnosed with PAH, our experienced staff will provide careful monitoring and treatment of your child to help you successfully manage the condition so your child can live a normal and fulfilling life. We treat patients from a few months old to young adulthood and will help in the transition to an adult clinic.
What It Is
PAH is a very serious disease that can occur as an isolated problem but is often associated with congenital heart disease and chronic lung disease. Its prevalence is becoming more common due to an increasing number of children who are successfully being treated and living longer with these diseases. PAH occurs when the bloods vessels that supply the lungs become constricted and their walls thicken, reducing the amount of blood they can carry to the lungs. This causes the heart to work harder. If the condition becomes severe enough, the heart may no longer be able to keep up with the demand for oxygen-rich blood needed to circulate to the body. Symptoms may include tiredness, shortness of breath, or even fainting with exertion.
Diagnosis
PAH is a progressive disease. While there is currently no cure for pulmonary hypertension, proper diagnosis and treatment can help slow, and some cases, halt the progression of the disease.
Diagnosis is achieved by performing a clinical history, an echocardiogram and/or a right heart catheterization. Finding the correct cause and severity of the disease will help your doctor determine the most effective treatment plan.
Echocardiography
An echocardiographic study, also called a cardiac ultrasound or echo, is a noninvasive procedure used to identify structural abnormalities in your child's heart. It can be performed on children of any age and size, including unborn babies at risk for having congenital heart disease.
The study uses very high frequency sound waves to form a moving, two-dimensional picture of your child's heart on a television screen. With this information, your doctor will also be able to evaluate how the heart muscle is functioning, record the speed of blood flow through the heart and determine blood pressure into the heart chambers.
In most cases, the pictures are taken using a noninvasive technique called transthoracic echocardiography. This involves the use of a small instrument called a transducer, which is gently placed on your child's chest. The pictures may also be taken from inside your child's esophagus using a more invasive technique called transesophageal echocardiography.
Cardiac catheterization
A cardiac catheterization is used to diagnose and sometimes treat many different heart conditions. It is a test that can help your cardiologist learn more about how your child's heart is working and to help determine the best treatment plan.
Cardiac catheterization involves inserting a catheter -- a thin, flexible, hollow tube into a vein or artery, usually in the groin. A local anesthetic numbs the skin and muscle around the entry area where the catheter is inserted. Neither an incision nor stitches is necessary. Once the catheter has been inserted into the blood vessel, the cardiologist uses a fluoroscope, which allows the doctor to guide the catheter into the different areas of the heart. The movement of the catheter within the heart is not painful, because the inner parts of the heart do not have nerve endings.
While the tube is in the heart, several procedures are done. These include recording blood pressures in the different heart chambers and blood vessels; evaluating oxygen content of the blood in each chamber of the heart; injecting a liquid dye through the catheter and filming X-ray "movies" called angiograms. These pictures enable the cardiologist to see most abnormalities inside the heart. In patients being evaluated for pulmonary hypertension, response to different medications is directly measured in the catheterization laboratory.
Treating PAH
The goal of treatment is to slow or even halt the progression of PAH thereby preserving the function of the right ventricle -- the pumping chamber to the arteries that deliver "blue blood" to the lungs to receive oxygen. Failure to treat PAH will result in gradual progression of the disease and can be fatal.
After diagnosis, your doctor will carefully select the medications or combination of medications and therapies that will be most effective in treating your child's condition. Your child may be treated with oxygen, agents to help your heart pump better, diuretics, anticoagulants (blood thinners), and medications to lower your PAH.
An echocardiogram and/or a right heart catheterization will be performed shortly after initiating medications to monitor their effect on your child. These tests will continue to be performed on a quarterly basis to monitor their effectiveness.
Ventilation
Ventilatory support is often a short-term therapy that is used on young infants born with premature lungs to allow their lungs to grow and mature. Adequate ventilation is needed to prevent the condition from becoming worse. Assisted ventilation is sometimes needed in older children as well. We collaborate closely with pulmonology doctors to ensure your child grows and thrives.
Medications
When the cause of PAH relates to pulmonary arterial disease, treatment involves oral and intravenous medications. These medications include:
Oral medications:
Intravenous medications:
Inhaled medications:
A diagnosis of PAH doesn't necessarily mean your child can't have an active, fulfilling life. However, he or she will need to consider certain measures and precautions. To better manage the disease you and your child will need to learn what types of factors can make it worse. These include:
- Smoking or second-hand smoke
- Respiratory infection
- High altitude
- Aerobic exercise
While children should be as active as physically possible, physical activity also can be associated with marked increases in pulmonary artery pressure. Thus, your child shouldn't participate in isometric exercises and activities that produce dangerous symptoms such as chest pain or dizziness. A supervised cardiopulmonary rehabilitation program may help promote conditioning.
Additional precautions are often taken with PAH patients. These include supplemental oxygen during air travel, antibiotic therapy for significant respiratory tract infections, pneumococcal pneumonia vaccine and yearly flu vaccines (since pneumonia can be very serious with PAH patients). Also avoid conditions in which the ambient oxygen concentration may be decreased, such as high altitude and travel in unpressurized airplane cabins.
Types of pulmonary hypertension
PAH has a number of causes. The cause of your child's PAH will help determine the most appropriate treatment. The most common causes include:
- Idiopathic (no known cause) pulmonary hypertension
- Familial pulmonary hypertension (inherited)
- Pulmonary hypertension associated with other conditions
- Pulmonary hypertension associated with congenital heart disease
- Pulmonary hypertension associated with lung disease
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