scholarly journals Prevalence of cardiac abnormalities in sickle cell disease identified using cardiac magnetic resonance imaging

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
M Bawor ◽  
R Kesse-Adu ◽  
K Gardner ◽  
P Marino ◽  
J Howard ◽  
...  
Keyword(s):  
Heart Failure ◽  
Sickle Cell Disease ◽  
Ejection Fraction ◽  
Cardiac Function ◽  
Sickle Cell ◽  
Cardiac Mri ◽  
Left Ventricular ◽  
Cell Disease ◽  
Cardiac Abnormalities ◽  
Atrial Enlargement

Abstract Background Sickle cell disease (SCD) affects thousands of individuals in the United Kingdom causing significant morbidity and mortality. Modern therapies have been successful in increasing life expectancy, however these patients have an increased risk of cardiovascular complications and the extent to which sickle cell disease affects cardiac function is not well understood. Cardiac magnetic resonance imaging (MRI) is the gold standard imaging modality for evaluating myocardial function. It is known that sickle cell patients can present with pulmonary hypertension, left ventricular diastolic dysfunction, and atrial enlargement however the prevalence of other cardiac abnormalities has not been sufficiently investigated with cardiac MRI. In addition, the European Society of Cardiology (ESC) updated their definition of Heart Failure in 2016 and therefore will need to be re-assessed in this population. Purpose To evaluate the prevalence of cardiac abnormalities in the sickle cell population using cardiac MRI and based on the recently updated diagnostic criteria. Methods We conducted a retrospective review including all patients with sickle cell disease at a large tertiary hospital in London, United Kingdom who had been referred for cardiac MRI between 2011 and 2019. Data was collected data on various measures of cardiac function including: left ventricular ejection fraction (LVEF), left ventricular hypertrophy, left and right atrial enlargement, regional wall motion abnormalities, valvular disease, myocardial scarring, and cardiac iron load. Results 82 patients and 123 cardiac MRI scans were reviewed in this study. 68% of patients were female and the average age at time of scan was 37 years. The average left ventricular ejection fraction was 57% (n=82). Cardiac abnormalities were identified in 60% of patients. The most common cardiac abnormalities reported were: valvular regurgitation (46%; n=28), left atrial enlargement (28%; n=19), right atrial enlargement (16%; n=11), left ventricular hypertrophy (11%; n=8), regional wall motion abnormalities (10%; n=7), and myocardial scar with late gadolinium enhancement (9%; n=7). 28% of the patients were diagnosed with Heart Failure; 11% of the patients satisfied the diagnostic criteria for HFpEF (Heart failure with preserved ejection fraction, n=9), 10% with HFrEF (Heart Failure with reduced ejection fraction, n=8), and 7% with HFmrEF (Heart Failure with mid-range ejection fraction, n=6). Conclusion Sickle cell disease affects cardiac function in the majority of patients resulting in numerous cardiac abnormalities. We have described the overall extent of these effects using data from cardiac MRI scans, which has not been commonly used thus far. This has implications for both the diagnosis and subsequent management of cardiac abnormalities in this population, and it can be used to further investigate and guide the development of targeted treatments for these patients. Funding Acknowledgement Type of funding source: None

scholarly journals Sickle cell disease: at the crossroads of pulmonary hypertension and diastolic heart failure

Heart ◽  
2019 ◽  
Vol 106 (8) ◽  
pp. 562-568 ◽  
Author(s):  
Katherine C Wood ◽  
Mark T Gladwin ◽  
Adam C Straub
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Sickle Cell Disease ◽  
Ejection Fraction ◽  
Diastolic Dysfunction ◽  
Sickle Cell ◽  
Diastolic Heart Failure ◽  
Left Ventricular ◽  
Cell Disease ◽  
Lv Diastolic Dysfunction

Sickle cell disease (SCD) is caused by a single point mutation in the gene that codes for beta globin synthesis, causing haemoglobin polymerisation, red blood cell stiffening and haemolysis under low oxygen and pH conditions. Downstream effects include widespread vasculopathy due to recurring vaso-occlusive events and haemolytic anaemia, affecting all organ systems. Cardiopulmonary complications are the leading cause of death in patients with SCD, primarily resulting from diastolic heart failure (HF) and/or pulmonary hypertension (PH). HF in SCD often features biventricular cardiac hypertrophy and left ventricular (LV) diastolic dysfunction. Among HF cases in the general population, approximately half occur with preserved ejection fraction (HFpEF). The insidious evolution of HFpEF differs from the relatively acute evolution of HF with reduced ejection fraction. The PH of SCD has diverse origins, which can be pulmonary arterial (precapillary), pulmonary venous (postcapillary) or pulmonary thromboembolic. It is also appreciated that patients with SCD can develop both precapillary and postcapillary PH, with elevations in LV diastolic pressures, as well as elevations in transpulmonary pressure gradient and pulmonary vascular resistance. Regardless of the cause of PH in SCD, its presence significantly reduces functional capacity and increases mortality. PH that occurs in the presence of HFpEF is usually of postcapillary origin. This review aims to assemble what has been learnt from clinical and animal studies about the manifestation of PH-HFpEF in SCD, specifically the contributions of LV diastolic dysfunction and myocardial fibrosis, in an attempt to gain an understanding of its evolution.

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.

scholarly journals Evaluation of cardiac function in patients with sickle cell disease with left ventricular global longitudinal strain

2020 ◽  
Vol 8 (1) ◽  
pp. 41-47
Author(s):  
Marielle Morissens ◽  
Tatiana Besse-Hammer ◽  
Marie-Agnès Azerad ◽  
Andre Efira ◽  
José Castro Rodriguez
Keyword(s):  
Sickle Cell Disease ◽  
Cardiac Function ◽  
Sickle Cell ◽  
Longitudinal Strain ◽  
Speckle Tracking Echocardiography ◽  
Myocardial Dysfunction ◽  
Global Longitudinal Strain ◽  
Left Ventricular ◽  
Biological Parameters ◽  
Cell Disease

AbstractBackground and ObjectivesThe importance of myocardial dysfunction in sickle cell disease (SCD) is currently debated. It is difficult to find a reliable index of function in patients with chronic overload as in SCD. Speckle tracking echocardiography, a new mean of evaluating cardiac function, might be a useful tool in SCD. It has been applied in many fields to detect early cardiac function deterioration, and it is less load dependent compared with other function parameters. Studies in patients with SCD are rare, and the results are conflicting. The present study aimed to determine whether left ventricular global longitudinal strain (LV-GLS) was abnormal in a population of adults with SCD and whether it was correlated with clinical or biological parameters.MethodsWe prospectively enrolled 37 patients and 34 age- and sex-matched healthy controls. Echocardiography was performed in patients and controls.ResultsWe found that the left ventricular diameter and mass were higher and the ejection fraction and longitudinal strain were lower in patients compared with controls. Diastolic dysfunction was uncommon. LV-GLS was abnormal in 21% of the patients. No correlation was observed between strain and clinical or biological parameters.ConclusionsWe concluded that LV-GLS could be a useful tool for evaluating these patients. However, the clinical impact of reduced LV-GLS remains to be determined.

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 Peritoneal dialysis versus hemodialysis in the management of sickle cell disease patients with endstage renal disease: an observational study

2021 ◽  
Vol 9 (2) ◽  
pp. 53-59
Author(s):  
Abdullah Alhwiesh ◽  
Ibrahiem Saeed Abdul-Rahman ◽  
Abdulla Al - Shehri ◽  
Amani Alhwiesh ◽  
Nadia Al- Audah ◽  
...  
Keyword(s):  
Peritoneal Dialysis ◽  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Renal Disease ◽  
Cardiac Function ◽  
Sickle Cell ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Cell Disease ◽  
Esrd Patients

There is paucity of studies that discussed the role of peritoneal dialysis (PD) in managing end stage renal disease (ESRD) in sickle cell disease (SCD) patients. The present study compares the outcome of SCD-ESRD patients treated with hemodialysis (HD) or PD. Sixty incident ESRD patients were allotted to HD, (HD group, n=40) or PD, (PD group, n=20). Causes and severity of renal injury were assessed at the time of initiating dialysis. The primary outcome was hospital mortality at 5 years, and secondary outcomes were infection rates, incidents of vasoocclusive crisis (VOC) and acute chest syndrome (ACS), response to erythropoietic agents and improvement of cardiac function. No statistically significant differences were observed between groups in regard to patients’ characteristics. The survival at 5 years was significantly better in the patients treated with PD when compared to HD (75.0% vs. 57.5%, p=0.026). Infectious complications (15% vs 35%, p<0.001), blood transfusion requirements (p < 0.001), VOC (15% vs. 42.5%, p<0.001) and ACS (10% vs. 27.5%, p<0.001) were significantly less in the PD group. Response to erythropoietic agents and improvement of left ventricular ejection fraction (LVEF) were significantly better in the PD group (p = 0.022 and p < 0.001, respectively). This study suggests that there are better outcomes with PD compared to HD in the treatment of SCD-ESRD patients with different dialysis modalities. Key Words: SCD, HD, PD, erythropoiesis, blood transfusion, VOC, ACS, sepsis, cardiac function, survival.

scholarly journals Left ventricular hypertrophy and diastolic dysfunction in children with sickle cell disease are related to asleep and waking oxygen desaturation

Blood ◽  
2010 ◽  
Vol 116 (1) ◽  
pp. 16-21 ◽  
Author(s):  
Mark C. Johnson ◽  
Fenella J. Kirkham ◽  
Susan Redline ◽  
Carol L. Rosen ◽  
Yan Yan ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Oxygen Saturation ◽  
Diastolic Dysfunction ◽  
Sickle Cell ◽  
Left Ventricular ◽  
Multivariable Logistic Regression Analysis ◽  
Cell Disease ◽  
Low Oxygen ◽  
Cardiac Abnormalities ◽  
Lv Mass

Abstract Premature death and cardiac abnormalities are described in individuals with sickle cell disease (SCD), but the mechanisms are not well characterized. We tested the hypothesis that cardiac abnormalities in children with SCD are related to sleep-disordered breathing. We enrolled 44 children with SCD (mean age, 10.1 years; range, 4-18 years) in an observational study. Standard and tissue Doppler echocardiography, waking oxygen saturation averaged over 5 minutes, and overnight polysomnography were obtained in participants, each within 7 days. Eccentric left ventricular (LV) hypertrophy was present in 46% of our cohort. After multivariable adjustment, LV mass index was inversely related to average asleep and waking oxygen saturation. For every 1% drop in the average asleep oxygen saturation, there was a 2.1 g/m2.7 increase in LV mass index. LV diastolic dysfunction, as measured by the E/E′ ratio, was present in our subjects and was also associated with low oxygen saturation (sleep or waking). Elevated tricuspid regurgitant velocity (≥ 2.5 m/sec), a measure of pulmonary hypertension, was not predicted by either oxygen saturation or sleep variables with multivariable logistic regression analysis. These data provide evidence that low asleep and waking oxygen saturations are associated with LV abnormalities in children with SCD.

Heart Function Disorders In Sickle Cell Disease

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 4815-4815
Author(s):  
Charlotte Heinen ◽  
Karin Fijnvandraat ◽  
Marjolein Peters ◽  
Henriette Heijboer ◽  
Nico Blom ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Left Ventricle ◽  
The Netherlands ◽  
Sickle Cell ◽  
Hematological Parameters ◽  
Left Ventricular ◽  
Ventricular Dilatation ◽  
Cell Disease ◽  
Cardiac Abnormalities ◽  
Left Ventricular Dilatation

Abstract Abstract 4815 Abstract Background: Sickle cell disease (SCD) is a hereditary anemia characterized by chronic hemolytic anemia and vaso-occlusive episodes, associated with a high susceptibility for infections and damage to all vital organs. Cardiologic effects of SCD are dilatation and hypertrophy of the left ventricle and pulmonary hypertension. The prevalence of these cardiac complications in children with SCD living in the Netherlands is unknown. The role of hemolysis in this process is still unclear. Therefore, the objectives of this study are: 1) to evaluate the prevalence of cardiac abnormalities in children with SCD living in the Netherlands, 2) to analyse the association between cardiac abnormalities and the severity of anemia and hemolysis. Methods: Cross-sectional study, including 92 children with SCD (genotype HBSS, HBSC or HBS-beta 0) treated at the Emma Children's Hospital AMC. None of the children had cardiac abnormalities unrelated to SCD. Echocardiograms and electrocardiograms were performed in a stable state. All cardiac values were corrected for BSA. Echocardiographic values 20% above or under the average of an age matched control group were considered abnormal. Laboratory hematological parameters were obtained within one year of the cardiac imaging. All statistical analyses were performed with SPSS 18 software, using Pearson correlations and T-tests. Forced-entry multivariate regression analysis was conducted with hematological parameters, age and SCD genotype as independent variables and cardiologic parameters as dependent variables. Results: The study group had a mean age of 13 years (SD 5,5) and contained 49 boys (53,3%). All children had a normal shortening fraction. Almost half of the children (39%) had a dilated left ventricular internal diameter in diastole and systole. Dilatation was inversely correlated with the Hb level. When analysed with multivariate analysis, both Hb and bilirubin were independently associated with left ventricular dilatation, suggesting that hemolysis deteriorates cardiac structure by a mechanism separate from the effect of anemia. There was no association between the left ventricular dilatation and age. Conclusion: Pediatric sickle cell patients have significant dilatation of the left ventricle, an abnormality that is significantly and independently associated with the severity of anemia and bilirubin levels, but in spite retain a normal shortening fraction. Disclosures: No relevant conflicts of interest to declare.

Left ventricular function by echocardiogram in children with sickle cell anaemia in Mumbai, Western India

2014 ◽  
Vol 25 (7) ◽  
pp. 1319-1325 ◽  
Author(s):  
Abhay Tidake ◽  
Pranil Gangurde ◽  
Anup Taksande ◽  
Ajay Mahajan ◽  
Pratap Nathani
Keyword(s):  
Sickle Cell Disease ◽  
Left Ventricular Function ◽  
Ventricular Function ◽  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Left Ventricular ◽  
Western India ◽  
Cell Disease ◽  
Cardiac Abnormalities ◽  
Doppler Study

AbstractIntroductionCardiovascular events and complications are the leading cause of mortality and morbidity in patients with sickle cell disease. Cardiac abnormalities occur frequently and at an early stage in sickle cell anaemia patients, despite being more evident in adulthood. Sickle cell anaemia patients are increasingly able to reach adulthood owing to improved healthcare, and may, therefore, suffer the consequences of chronic cardiac injury. Thus, the study of cardiac abnormalities is essential in childrenObjectiveThe aim of this study was to determine the echocardiographic changes in left ventricular function in children suffering from sickle cell disease in Mumbai, Western India.MethodsThe study comprised of 48 cases of sickle cell anaemia and 30 non-anaemic controls with normal haemoglobin and electrophoresis pattern. M-mode, two-dimensional, and Doppler echocardiographic measurements of patients and controls were performed according to the criteria of the American Echocardiography Society.ResultsOn Doppler study, the A wave height was increased and the E/A ratio was decreased, whereas the deceleration and isovolumetric relaxation times were prolonged, which is typically seen in slowed or impaired myocardial relaxation (p<0.001). Although chamber dilatations were present, echocardiographic parameters showed no statistically significant correlation with severity of anaemia and age among the sickle cell patients.ConclusionsWe conclude that the increased left ventricular stiffness, compared with controls, might be due to fibrosis related to ischaemia caused by SS disease in addition to wall hypertrophy.

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