Sickle Cell Disease-Associated Pulmonary Arterial Hypertension

Pulmonary hypertension (PH) is known to affect between 6 and 10 percent of people with sickle cell disease.

At the UPMC Comprehensive Pulmonary Hypertension Program, we place a priority on screening our PH patients for sickle cell disease to avoid any additional complications.

Diagnosing Pulmonary Hypertension Due to Sickle Cell Disease

We work very closely with the UPMC Adult Sickle Cell Program, where patients usually obtain the initial screening echocardiogram.

Based on its results, patients are then:

• Referred to our Comprehensive Pulmonary Hypertension Program
• Evaluated with a history and physical exam
• Ordered for a repeat echocardiogram, if necessary

Using certain parameters on the echocardiogram, patients are considered to be at a low, medium, or high risk of having pulmonary hypertension.

Based on any suspicion from the echocardiogram, a patient may require a right heart catheterization to make the diagnosis of PH.

Treating Pulmonary Hypertension Due to Sickle Cell Disease

While significant research has been performed, there are no treatments specific to sickle cell disease-associated PH.

Patients with this condition may first maximize medical therapy for sickle cell disease and then apply treatments that have worked in other populations with PH.

Doctors usually use the following interventions for patients with sickle cell disease-associated pulmonary hypertension in this order:

1. Identification and treatment of any condition that may affect the lungs and worsen PH, such as:
• Asthma
• Pulmonary embolism
• Obstructive sleep apnea
2. Optimization of management of sickle cell disease with hydroxyurea and/or blood transfusions to minimize the number of pain and acute chest syndrome episodes.
3. Pulmonary vasodilator therapy with PH medications.
4. Lung transplant surgery.

While lung transplant can carry risks, patients can be comforted by the breadth of experience and outcomes of the UPMC Lung Transplant Program team. UPMC performed the first lung transplant in a patient with sickle cell disease-associated pulmonary hypertension, with an excellent surgical outcome.

Read stories of our UPMC lung transplant patients.

There are many complexities to lung transplant that require an experienced transplant center, as well as special preparation prior to listing. We recommend that patients seek early referral for transplant evaluation, especially those with worsening sickle cell disease-associated pulmonary hypertension.

Make an Appointment for Sickle Cell Disease-associated PH Today

To schedule an appointment at the UPMC Comprehensive Pulmonary Hypertension Program, contact us at:

• 1-877-PH4-UPMC, toll-free
• PHprogram@upmc.edu