Evaluation of right heart function in sickle cell patients

Objective: Sickle cell disease (SCD) is an inherited hemolytic anemia caused by mutations in hemoglobin subunit beta. Pulmonary hypertension and right heart failure are common in SCD and responsible for early mortality.Cardiac failure as a result of myocardial fibrosis causes dismal outcome in SCD. In this study, our aim was to determine right ventricular function as assessed by echocardiography, and also to investigate any quantitative changes in collagen tissue characteristic of myocardial fibrosis in patients with SCD. Materials and Methods: Fifty-seven patients with sickle cell disease and 56 age and gender matched healthy controls were enrolled in the study. Clinical and laboratory parameters and sickle-cell end-organ manifestations were evaluated in SCD patients and control group accordingly. Conventional and tissue Doppler echocardiography was performed. ELISA was used for the determination of serum levels of NT-proBNP, PICP, PIIINP and Gal-3. Statistical analysis were performed using IBM SPSS Statistics Version 21.0. Results: Seven (%12) of the patients with sickle cell disease had HbS-beta disease. While LVEF was preserved, right atrial diameter (RAD), right ventricular myocardial performance index (RVMPI), fractional area change (FAD) and tricuspid regurgitation velocity (TRV) measurements were found significantly higher in SCD patients compared to healthy controls (p <0.05). Sickle patients had higher NT-proBNP, Gal-3 and PICP levels compared to healthy controls (p<0.05). Conclusion: Doppler echocardiography in combination with circulating biomarker measurements seems to be an attractive option to evaluate cardiac dysfunction in SCD patients. It is likely evident that prolonged hydroxyurea therapy could prevent serious consequences of sickle cell disease including cardiac dysfunction, despite a minimal benefit observed in cardiac parameters with hydroxyurea in our study. Keywords: Sickle cell disease, pulmonary hypertension, Gal-3, NT-proBNP, PICP, PIIINP