Pulmonary Hypertension

What is pulmonary hypertension?

Pulmonary hypertension is high blood pressure in the arteries inside the lungs. This makes it harder for the right side of the heart to pump blood into the lungs. Over time, this can cause the right side of the heart to become enlarged and lead to heart failure.

There are several types of pulmonary hypertension, and they are grouped by the cause of the disease.

What are the signs and symptoms pulmonary hypertension?

Symptoms of pulmonary hypertension in children may include:

• Shortness of breath
• Rapid heart rate
• Dizziness
• Fainting
• Swelling in the hands or legs

What causes pulmonary hypertension?

There are five major types, called groups, of pulmonary hypertension, based on the cause of the disease:

Group 1: Pulmonary arterial hypertension. This type occurs when the arteries in the lungs become narrow or thickened. It can be caused by another heart or lung disease, or can have no apparent cause.

Group 2: This type of pulmonary hypertension is caused when the left side of the heart is failing. This causes blood flow to back up, leading to increased pressure in the lungs. It can be caused by several types of heart disease, including mitral stenosis, cardiomyopathy and pulmonary vein stenosis.

Group 3: This type of pulmonary hypertension is caused by lung disease, such as interstitial lung disease, sleep apnea and cystic fibrosis.

Group 4: This type is caused by blood clots in the lungs.

Group 5: This group includes all other causes of pulmonary hypertension.

How is pulmonary hypertension treated?

There is no cure for pulmonary hypertension. Treatment will focus on relieving symptoms and slowing the course of the disease. The treatment your child receives will depend on their symptoms and specific type of disease and whether they have another heart or lung disease. Your pediatric pulmonology and cardiology teams at Arkansas Children's are experienced in treating pulmonary hypertension in children and will work with you to determine the best course of treatment for your child. Some options may include:

• Oxygen to help with breathing and relax blood vessels in the lungs
• Medications to help relax the blood vessels in the lungs, reduce clotting, or reduce the amount of fluid in the body