scholarly journals Echocardiographic phenotypes of patients with sickle cell disease. An unsupervised analysis based on etendard cohort

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
T D"humieres ◽  
J Inamo ◽  
S Deswarte ◽  
T Damy ◽  
G Loko ◽  
...  
Keyword(s):  
Cluster Analysis ◽  
Cardiac Output ◽  
Sickle Cell Disease ◽  
Diastolic Function ◽  
Sickle Cell ◽  
Left Ventricular ◽  
Integrative Approach ◽  
Cell Disease ◽  
Clinical Profiles ◽  
Cluster 2

Abstract Funding Acknowledgements Type of funding sources: Public grant(s) – National budget only. Main funding source(s): PHRC Backgroung Echocardiography is the cornerstone in the diagnosis of cardiopulmonary involvement in sickle cell disease (SCD). However, given the unique pathophysiology of SCD associating high cardiac output, and various degrees of peripheral vasculopathy, differentiate the pathological from the physiological using echocardiography can be particularly challenging. Purpose This study sought to link cardiac phenotypes in homozygous SCD patients with clinical profiles and outcomes using cluster analysis. Methods We analyzed data of 379 patients with a sufficient echographic dataset included in the French Etendard Cohort, a prospective cohort initially designed to assess the prevalence of pulmonary hypertension. A cluster analysis was performed on echocardiographic variables, and the association between clusters and clinical profiles and outcomes was assessed. Results Three clusters were identified. Cluster 1 (N = 122) patients had the lowest cardiac output, only mild left cavities remodeling, diastolic dysfunction, and high tricuspid regurgitation velocity (TRV). They were predominantly female, as old as cluster 2, and displayed the most severe functional limitation. Cluster 2 (N = 103) patients had the highest cardiac output, left ventricular mass and a severely dilated left atrium. Diastolic function and TRV were similar to cluster 1. These patients had a higher blood pressure and a severe hemolytic anemia. Cluster 3 (N = 154) patients had mild left cavities remodeling, the best diastolic function and the lowest TRV. They were younger patients with the highest hemoglobin and lowest hemolytic markers. Right heart catheterization was performed in 94 patients. Cluster 1 gathered the majority of precapillary PH while cluster 2 gathered postcapillary PH and no PH was found in cluster 3. After a follow-up of 9.9 years (IQR: 9.3 to 10.5 years) death occurred in 38 patients (10%). Clusters 2 had the worst prognosis with 18% mortality rate vs. 12% in cluster 2 and 5% in cluster 1 (P log-rank = 0,02). Results are summarized in the central illustration. Conclusions Cluster analysis of echocardiographic variables identified 3 phenotypes among SCD patients, each associated with different clinical features and outcome. These findings underlines the necessity to rethink echocardiographic evaluation of SCD patients, with an integrative approach based on simultaneous evaluation of TRV along with left cavities remodeling and diastolic parameters. Abstract Figure.

scholarly journals Assessment of Cardiac Abnormalities in Sickle Cell Disease Patients Using Echocardiography

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 4837-4837
Author(s):  
Osama Mukarram ◽  
Gian Lima ◽  
Samuel Crooks ◽  
Min Jung Kim ◽  
Agnes S Kim
Keyword(s):  
Heart Failure ◽  
Sickle Cell Disease ◽  
Diastolic Function ◽  
Sickle Cell ◽  
Left Atrial ◽  
Severe Form ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Healthy Controls ◽  
Cell Disease

Background: Sickle Cell Disease (SCD) is characterized by chronic anemia and recurrent ischemia-reperfusion episodes that contribute to high output heart failure. The effects of SCD on the heart are significantly underrecognized. Methods: SCD patients who underwent echocardiography between March 2016 and March 2018 were retrospectively analyzed. Patients with reduced Left Ventricular Ejection Fraction (LVEF) and valvular heart disease were excluded. Cardiac chamber size, systolic and diastolic function parameters, and LV and RV strain were compared between hemoglobin SS (most severe form of SCD) and SC (less severe form) subtypes and against healthy controls. Wilcoxon signed rank test was used for statistical analysis. Results: SS patients (n = 48, mean age 31.9) had lower mean hemoglobin 8.9 g/dl vs 11.3 g/dl (p < 0.001) and hematocrit 25.8% vs 31.4% (p = 0.008) and higher LDH 437 IU/L vs 258 IU/L (p < 0.001) compared to SC patients (n = 11, mean age 34.4). Both SS and SC patients had worse diastolic function compared to healthy controls: higher E velocity 98.9 cm/s (SS), 86.4 cm/s (SC), 76.4 cm/s (control) (SS vs control, p < 0.01; SC vs control, p < 0.05) and higher E/A ratio 1.76 (SS), 1.59 (SC), 1.15 (control) (SS vs control, p < 0.001; SC vs control, p < 0.01). SS patients had larger indexed left atrial volume compared to SC patients (39.3 ml/m2 vs 28.4 ml/m2, p = 0.007). There was no significant difference in LVEF, left ventricular global longitudinal strain, or right ventricular strain between SS and SC subtypes compared to healthy controls. Furthermore, SS patients with a serum LDH > 500 IU/L had higher E/e' ratio (11.3 vs 7.2, p=0.001) and larger indexed Left Ventricular End Diastolic Volume (LVEDVi) (80.8 ml/m2 vs 53.4 ml/m2, p=0.002) compared to SS patients with LDH < 500 IU/L. Conclusion: SCD genotype adversely determines the degree of cardiac dysfunction in patients with SCD. LVEDVi, left atrial size, E velocity, E/A ratio, and E/e' ratio may serve as useful echocardiographic parameters to follow in this patient population. Serum LDH has been associated with poor clinical outcomes in patients with SCD, and as demonstrated by our study, it also portends worsening cardiac function in this population at high risk for heart failure. Figure Disclosures No relevant conflicts of interest to declare.

scholarly journals Topic: Echocardiographic Evaluation of Left Ventricular Systolic and Diastolic Function in Nigerians with Sickle Cell Disease

OALib ◽  
2018 ◽  
Vol 05 (05) ◽  
pp. 1-8
Author(s):  
Hadiza Saidu ◽  
Abdulwahab Kabir ◽  
Jamila A. Yau ◽  
Ahmad M. Yakasai ◽  
Umar Abdullahi ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Diastolic Function ◽  
Sickle Cell ◽  
Left Ventricular ◽  
Cell Disease ◽  
Echocardiographic Evaluation ◽  
Systolic And Diastolic Function

Abstract 65: Anemia-related Heart Failure in Sub-saharan African Sickle Cell Disease Patients

2017 ◽  
Vol 121 (suppl_1) ◽  
Author(s):  
Adebayo C Atanda ◽  
Yahya Aliyu ◽  
Oluwafunmilayo Atanda ◽  
Aliyu Babadoko ◽  
Aisha Suleiman ◽  
...  
Keyword(s):  
Heart Failure ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Systolic Pressure ◽  
Left Ventricular ◽  
Hemoglobin Level ◽  
Sub Saharan Africa ◽  
Cell Disease ◽  
Jet Velocity ◽  
Sub Saharan

Introduction: Anemia has been implicated in heart failure. Existing literatures, involving predominantly African-Americans, suggests that Sickle Cell Disease (SCD) maybe linked to various cardiovascular complications including pulmonary hypertension and left venticular dysfunction. Peculiarly, our study involves exclusively Sub-Saharan population. Method: We conducted a cross sectional observational study of 208 hydroxyurea-naive consecutive SCD patients aged 10-52 years at steady state and 94 healthy non-matched controls who were studied in an out patient clinic in Sub-Saharan Africa. SCD patients were required to have electrophoretic or liquid chromatography documentation of major sickling phenotypes. Control group was required to have non-sickling phenotypes. Cardiac measurements were performed with TransThoracic Echo according to American Society of Echocardiography guidelines. Hemoglobin level was also obtained. Results: Hemoglobin level in SCD group (8.5+/- 1.5) was significant (P<0.001) compared to control (13.8+/- 1.7). Although SCD group had significantly higher values of left ventricular (LV) size, there was no qualitative evidence of LV dysfunction. SCD group had higher values of Ejection Fraction but not statistically significant. There was no evidence of LV wall stiffening to impair proper filling in SCD group, with the ratio of early to late ventricular filling velocities, E/A ratio elevated (1.7+/-0.4 compared to 1.6+/- 0.4; P=0.010). Right ventricular systolic pressure was determined using the formula of 4x Tricuspid Reugurgitant jet (TRV) square as an indirect measurement of Pulmonary arterial systolic pressure. SCD patients had significantly higher mean±SD values for tricuspid regurgitant jet velocity than did the controls (2.1±0.6 vs. 1.8±0.5; p= 0.001). Within the SCD group, there was no clear pattern of worsening diastolic function with increased TRV. Furthermore, E/A had a significant positive relationship with jet velocity in bivariate analysis (R=0.20; P=0.013). Conclusions: We were unable to demonstrate existence of anemia-associated left ventricular dysfunction in Sub-Saharan African with SCD. Further studies is required to highlight the reason behind this finding.

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