Sickle Cell Disease and Pulmonary Hypertension

These instructions are for Children's Hospital of Philadelphia (CHOP) patients with sickle cell disease to learn more about pulmonary hypertension.

Important information:

Pulmonary hypertension (PH) is when the pressure in the blood vessels that carry blood from your heart to your lungs is higher than normal. Sometimes it is called "high blood pressure of the lungs."

One in 3 older children and adults with sickle cell disease also have pulmonary hypertension. It is a very serious condition.

A screening test can help determine who is at risk for developing pulmonary hypertension. The screening test is an ultrasound of the heart, also known as echocardiogram (ECHO). All CHOP Sickle Cell Center patients 18 years or older will be screened.

Signs and symptoms of pulmonary hypertension include:

• Chest pain

• Fatigue with exercise

• Shortness of breath

Patient instructions:

• Take daily medicine for sickle cell disease as prescribed.

• Schedule the screening test as recommended by your sickle cell team.

• If the result is positive, the team will refer you to a cardiologist, a heart specialist, for further studies and treatment if needed.

Contact your CHOP hematology team with questions or concerns.

Division of Hematology

215-590-3535

Sickle Cell Center

For non-urgent issues, send your team a message in the MyCHOP portal.

Reviewed on June 23, 2023, by Alexandra Kaspin, RN, MSN