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Published on: December 22, 2023
Detection of Subclinical Cardiac Dysfunction in Patients With Sickle Cell Disease Using Speckle-Tracking
Min-Jung Kim1, Grace Lee2, Gian Lima1
1Calhoun Cardiology Center, School of Medicine, University of Connecticut School of Medicine, Farmington, Connecticut.
Insights
Sickle cell disease (SCD) significantly impacts cardiac structure and function, leading to impaired left and right ventricular strain. These cardiac changes in SCD patients warrant further investigation for clinical outcomes.
Area of Science:
- Cardiology
- Hematology
- Medical Imaging
Background:
- Sickle cell disease (SCD) is linked to chronic anemia and ischemia-reperfusion, potentially causing heart failure.
- The specific effects of SCD on cardiac structure and function are not well understood.
Purpose of the Study:
- To investigate cardiac structural and functional differences in patients with sickle cell disease (SCD) compared to healthy controls.
- To identify echocardiographic parameters affected by SCD.
Main Methods:
- Retrospective analysis of clinical and echocardiographic data from 66 patients with hemoglobin SS SCD (SCD-SS) and 28 healthy controls.
- Exclusion of patients with pre-existing heart conditions, diabetes, hypertension, or lung disease.
- Echocardiographic assessment included left atrial (LA) volume index, E/e', e', LA reservoir strain, LV volumes, LV global longitudinal strain, RV global longitudinal strain, tricuspid regurgitation velocity, and right atrial area.
Main Results:
- SCD-SS patients exhibited significantly higher LA volume index, LV end-diastolic and end-systolic volumes, and tricuspid regurgitation velocity compared to controls.
- Patients with SCD-SS showed reduced LA reservoir strain, LV global longitudinal strain, and RV global longitudinal strain.
- Lower hemoglobin and hematocrit levels correlated with increased LA volume index and LV volumes in SCD-SS patients.
Conclusions:
- Patients with SCD-SS demonstrate significant alterations in cardiac chamber size and impaired myocardial strain in the left ventricle, right ventricle, and left atrium.
- These echocardiographic findings highlight potential subclinical cardiac dysfunction in SCD.
- Further research is necessary to determine the clinical significance and prognostic implications of these cardiac changes in SCD.
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 underinvestigated. 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 with healthy controls (n = 28), patients with SCD-SS (n = 66) had a significantly higher left atrial (LA) volume index (35.7 vs 23.9 ml/m², p <0.001) and average E/e' (7.4 vs 6.5, p = 0.003) but 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). Patients with SCD-SS had higher LV end-diastolic (132.5 vs 104.1 ml, p <0.001) and LV end-systolic volumes (51.0 vs 43.8 ml, p = 0.017) with reduced LV global longitudinal strain (17.6% vs 20.0%, p <0.001). In addition, patients with SCD-SS showed reduced right ventricular (RV) global longitudinal strain (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 greater in SCD-SS. Hemoglobin and hematocrit negatively correlated with LA volume index, average E/e', LV end-diastolic and LV end-systolic volumes. In conclusion, patients with SCD-SS had notable differences in cardiac chamber size and impaired LV, RV, and LA strain compared with healthy controls. Further investigations are needed to assess the impact of these variables on SCD clinical course and prognosis.
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