Abstract

Sickle cell disease (SCD) is characterized by chronic anemia and recurrent ischemia-reperfusion episodes, which can lead to high output heart failure. The impact of SCD on cardiac structure and function remains under-investigated. We conducted a single-institution retrospective analysis of clinical and echocardiographic data from patients with hemoglobin SS SCD (SCD-SS) between January 2016 and June 2022. Patients with known heart failure, left ventricular (LV) ejection fraction <50%, moderate or severe valvular heart disease, congenital heart disease, established coronary artery disease, diabetes mellitus, hypertension, or coexistent lung disease were excluded. Compared to healthy controls (HC; n=28), SCD-SS patients (n=66) had significantly higher left atrial (LA) volume index (LAVi) (35.7 vs. 23.9 mL/m², p<0.001) and average E/e' (7.4 vs. 6.5, p=0.003), while having lower average e' (12.3 vs. 13.6 cm/s, p=0.047) and LA reservoir strain (32.9% vs. 42.4%, p<0.001). SCD-SS patients had higher LV end-diastolic volume (LVEDV) (132.5 vs. 104.1 mL, p<0.001) and LV end-systolic volume (LVESV) (51.0 vs. 43.8 mL, p=0.017) with reduced LV global longitudinal strain (GLS) (17.6% vs. 20.0%, p<0.001). Additionally, SCD-SS patients showed reduced right ventricular (RV) GLS (19.7% vs. 22.8%, p<0.001) in the setting of normal RV tricuspid annular plane systolic excursion. Maximal systolic tricuspid regurgitation velocity (231 vs. 202 cm/s, p<0.001) and right atrial area (16.6 vs. 12.8 cm², p<0.001) were statistically higher in SCD-SS. Hemoglobin and hematocrit negatively correlated with LAVi, average E/e', LVEDV, and LVESV. In conclusion, SCD-SS patients had notable differences in cardiac chamber size and impaired LV, RV, and LA strain compared to healthy controls. Further investigations are needed to assess the impact of these variables on SCD clinical course and prognosis.