Cardiovascular Consequences of Sickle Cell Disease

Area of Science:

• Cardiology
• Hematology
• Pulmonology

Background:

• Sickle cell disease (SCD) is an inherited blood disorder with potential severe cardiovascular and respiratory complications.
• These complications include arrhythmias, pulmonary hypertension (PH), and cardiomegaly, impacting adult patient health.
• Adult SCD patients are at risk for PH and arrhythmias, which are frequently underdiagnosed.

Purpose of the Study:

• To investigate the prevalence and risk factors of pulmonary hypertension (PH) and arrhythmias in adult patients with sickle cell disease (SCD).

Main Methods:

• Retrospective analysis of medical records for 517 adult SCD patients (aged 15+) from 2009-2021.
• Echocardiograms and electrocardiograms were assessed using ESC/ERS guidelines for PH risk and arrhythmia detection.
• Multivariate logistic regression identified independent risk factors for PH and arrhythmias.

Main Results:

• 1.4% of patients had arrhythmias, 3.7% moderate PH risk, and 3.3% high PH risk.
• Chronic kidney disease (CKD) was a significant independent risk factor, increasing PH odds by 26.4 times and arrhythmia odds by 15.36 times.
• Other key risk factors for PH included liver cirrhosis and pre-existing cardiac conditions; arrhythmias were associated with CKD and cardiac conditions.

Conclusions:

• Adult SCD patients are at significant risk for PH and arrhythmias, often undiagnosed.
• CKD, liver cirrhosis, and pre-existing cardiac conditions are key risk factors.
• Multidisciplinary care and prospective studies are recommended for early detection and improved outcomes in hemoglobinopathy patients.