Pulmonary hypertension in sickle cell disease

Area of Science:

• Cardiology
• Hematology
• Pulmonology

Background:

• Sickle cell disease (SCD) is a genetic disorder with pulmonary hypertension (PH) as a debilitating complication.
• SCD-associated PH is often post-capillary but can be pre-capillary due to various interrelated mechanisms.
• Accurate diagnosis and classification of PH are essential for effective management.

Purpose of the Study:

• To highlight the diagnostic pathway for SCD-associated PH.
• To discuss current and emerging treatment strategies for PH in SCD patients.
• To emphasize the need for a multidisciplinary approach in managing this complex condition.

Main Methods:

• Screening symptomatic patients with echocardiography to identify those needing right heart catheterization.
• Utilizing right heart catheterization for definitive diagnosis and PH categorization.
• Assessing underlying mechanisms of PH for personalized treatment.

Main Results:

• Echocardiography and right heart catheterization are crucial for diagnosing and categorizing SCD-associated PH.
• Treatment involves managing the underlying SCD with hydroxyurea or transfusions.
• Efficacy data for PAH-specific therapies in SCD are limited, requiring expert assessment.

Conclusions:

• Personalized treatment of SCD-associated PH depends on identifying causative mechanisms.
• Expert centers should manage PAH-specific therapies based on individual benefit-risk assessments.
• A multidisciplinary approach involving SCD and PH specialists is vital for optimal patient care.