Researchers reported that cardiac magnetic resonance (CMR) may be useful in identifying patients with sickle cell disease (SCD) who are at a higher risk of developing cardiopulmonary and vascular diseases, according to a study published in Annals of Hematology.  

SCD is an inherited disorder of hemoglobin production that can result in reduced quality of life. However, due to advancements in diagnosis and treatment, many patients receive care from infancy; this includes penicillin prophylaxis for pneumococcal infection and the early initiation of red blood cell transfusion and hydroxyurea therapy when appropriate. 

“The two clinical hallmarks of SCD, hemolysis and vaso-occlusive crises with repeated episodes of ischemia and reperfusion, strongly contribute to cardiovascular involvement,” the authors of the study wrote. 

Read more about SCD etiology 

As a result of these manifestations of SCD, microvascular dysfunction occurs, triggering a chronic state of inflammation and widespread vasculopathy. The result is multi-organ damage, especially to the heart and the lungs. The chronic anemic state in SCD results in compensatory mechanisms to increase blood volume, which often results in the dilation of all cardiac chambers, causing cardiac wall stress and hypertrophy. 

CMR is a powerful tool for evaluating structural and functional dysfunction in the myocardium. In addition, it is non-invasive, fast, and is considered the gold standard for assessing biventricular size and function. The authors of the study hence sought to assess the role of CMR in predicting cardiovascular complications in patients with SCD. 

The research team recruited patients with SCD (n=102) from the Myocardial Iron Overload in Thalassemia Network, a collaborative project that includes patients with various hemoglobinopathies. All MRI examinations were performed using a clinical 1.5 T scanner. The research team assessed the presence of myocardial fibrosis using late gadolinium enhancement images. 

The researchers discovered that a reduction in left ventricular ejection fraction and an increase in right ventricular mass index as detected via CMR held statistically significant prognostic value in patients with SCD. 

“To the best of our knowledge, this is the first study exploring the value of multiparametric CMR, including [right ventricular] mass assessment, in the prognostic evaluation of SCD patients,” the authors of the study wrote. 

Reference

Meloni A, Pistoia L, Quota A, et al. Prognostic value of multiparametric cardiac magnetic resonance in sickle cell patientsAnn Hematol. Published online December 2, 2022. doi:10.1007/s00277-022-05057-6