Pediatric Pulmonary Arterial Hypertension

What is Pulmonary Arterial Hypertension?

Pulmonary arterial hypertension (PAH) relates to high blood pressure in the lung arteries.

PAH occurs when the arteries in the lungs become narrowed, thickened, or stiff. The right side of the heart must work harder to push blood through these compromised arteries. Over time, the heart may lose its ability to pump enough blood through the lungs to meet the body’s needs.

There are different types of PAH, which can begin in childhood or adulthood.

Idiopathic PAH and Heritable PAH

Idiopathic PAH (IPAH) is PAH that occurs without a clear cause. IPAH is relatively rare.

HPAH can be passed on from parent to child. Research suggests that genetic factors play a role in IPAH about 35% of the time.

Genetic counseling can help parents assess their risk for passing on certain health conditions, like pulmonary hypertension.

Pulmonary Hypertension in Children

With any kind of pulmonary hypertension, you may notice several symptoms in children, such as:

• Shortness of breath or hyperventilating
• Fatigue
• Fainting spells
• Lightheadedness or dizziness
• Coughing up blood
• Poor appetite
• Poor growth
• Rapid heart rate

Naturally, these signs and symptoms could indicate a wide range of conditions, so it’s important to speak with your pediatrician about any and all concerns. If a heart or lung condition is suspected, experts in pediatric pulmonary hypertension can evaluate the child’s condition.

Diagnosing IPAH and HPAH in Children

The multidisciplinary pulmonary hypertension team at Riley at IU Health diagnoses pediatric PAH using a variety of screenings. These may include:

• Blood tests: These may be used to rule out certain diseases or to screen for a condition that may cause PAH secondarily.
• Echocardiogram: An echocardiogram captures a moving image of the child’s heart as it works. The doctor can use this to locate defects such as narrow or leaking valves, holes in the heart walls, fluid around the heart, or other issues.
• Electrocardiogram (EKG): In this simple, painless test, a technician attaches electrodes to the child’s skin. A machine reads the heart’s electrical activity and can detect problems with the heartbeat.
• Cardiac catheterization: A small, flexible tube is threaded through a vein in the arm, upper thigh, or neck. Special dye is injected to allow the doctor to see how the blood flows through the heart and blood vessels. This test can also measure pressure inside the heart to determine if an opening is causing blood to seep from one side of the heart to the other.
• Sleep studies: During a sleep study, clinicians will monitor your child’s breathing, brain activity and vital signs. The results may help determine whether a heart or lung condition is interfering with their sleep.
• Chest x-ray: X-rays can show whether the heart is enlarged or the lungs have extra fluid in them.
• Pulmonary function testing: This may include a single test or a series of tests, such as spirometry, lung volumes, diffusion testing, respiratory muscle strength, or treadmill testing.
• Six-minute walk: In children old enough to walk, this simple test can tell doctors how well oxygen is moving through their blood. The child will walk at a normal pace for six minutes wearing a pulse oximeter.
• Ventilation/perfusion scan (V/Q): This diagnostic tool tests for blood clots in the lungs by looking at how air and blood flow to the lungs. The doctor will review the resulting images to evaluate the health of the lungs.
• Ultrasound: Your child's doctor may perform a chest ultrasound to see the heart, lungs and the area surrounding them (pleural cavity).
• Genetic testing: A cardiovascular genetic counselor will obtain a detailed family history. They will collect genetic testing, provide education about the diagnosis, and make suggestions for future medical care of your child. They may also discuss health risks and recommendations for other family members.

Outlook for Children with IPAH

Thanks to recent advances in cardiopulmonary medicine, children with IPAH can often enjoy a good quality of life. Some children with severe cases may be candidates for a lung transplant or other surgical procedure.