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 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.

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 GBT440 Increases Hematocrit and Improves Biventricular Function in Berkeley Sickle Cell Disease Mice

2020 ◽  
Vol 143 (3) ◽  
Author(s):  
Ryan Gassner ◽  
David Schreier ◽  
Timothy Hacker ◽  
Diana M. Tabima ◽  
Naomi Chesler
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Stroke Volume Index ◽  
Left Ventricular ◽  
Volume Index ◽  
Left Ventricular Ejection ◽  
Cell Disease ◽  
Ventricular Ejection Fraction ◽  
Blood Disorder ◽  
Biventricular Function

Abstract Sickle cell disease (SCD) is a hereditary blood disorder affecting millions of people in which red blood cells (RBCs) become sickled and lyse easily driven by polymerization of hemoglobin. Chronically, SCD causes anemia and biventricular dysfunction. GBT440 is an experimental treatment for SCD that prevents hemoglobin polymerization. We hypothesized that 17-month-old Berkeley SCD mice treated with GBT440 would have increased hematocrit (Hct) and better biventricular function compared to vehicle treated SCD mice. Our results demonstrate that 3 weeks of GBT440 treatment eliminated chronic anemia, increased left ventricular ejection fraction (LVEF) and stroke volume index, and improved right ventricular function. Overall, our findings support a therapeutic effect of GBT440 in vivo in a small animal model of SCD. Next steps in investigating mechanisms of improved cardiac function are warranted.

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

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 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

Manual Erythro-Exchange (MEEX) to Prevent Complications of Sickle Cell Disease in Patients Unresponsive to Hydroxyurea: A Long-Term Follow-up

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 4815-4815
Author(s):  
Gianluca Forni ◽  
Manuela Balocco ◽  
Laura Terenzani ◽  
Paola Carrara
Keyword(s):  
Sickle Cell Disease ◽  
Iron Overload ◽  
Sickle Cell ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Long Term Follow Up

Abstract Background: Hydroxyurea (HU) therapy reduces considerably mortality and morbility in sickle cell disease (SCD). In about 10–25% of adult patients HU is ineffective therefore other therapeutic options such as chronic red cell transfusions (CRT) or erythroexchange (EEX) are needed. From 1981 to 2007 in our Center 40 patients affected by homozygous sickle cell (S-S) and sickle-β0 thalassemia (S/β0) with a previous history of major events, pregnancy or prior to surgery were treated with periodic Manual Erithroexchange (MEEX). 1133 MEEX procedures were performed with only one case of immunization in a pregnant woman. Consequentely MEEX resulted to be safe and efficacious in preventing SCD manifestations as well as significantly inexpensive. Even if, since 1995 HU has been accepted as first line therapy, seven out of 40 patients, unresponsive to HU treatment, had been continuing on a program of periodic EEX. On the basis of our own experience, we decided to use manual EEX instead of automatic one. We retrospectively report the data referring to a long-term follow-up (11–26 yrs, medium 16,5 yrs) of these 7 patients. Methods and Patients: MEEX always started with an infusion of a 500 ml Ringer lactate solution followed by a phlebotomy (400–600 ml depending on weight and HbS level); autologous plasma derived from drawn blood centrifugation was re-infused to the patient. Thereafter a second phlebotomy was performed as previously described. Finally, depending on Hb level, each patient received two or three packed, Rh matched, leuko-filtered and plasma-depleted red cells units. A single peripheral venous access was required. The gap between each MEEX ranged from 45 to 90 days in order to maintain HbS levels below 60%. The seven patients (3 males and 4 females) affected by S/beta° (6 pts) and S-S (1pts), at the beginning of the MEEX program were aged 8 to 26 years (mean 14yrs). They were enrolled because of acute chest syndrome (2 pts) and &gt;3 painful crises/yr (5pts). Patients underwent a median of 109 (61–180) MEEX procedures. Results: In the seven observed patients no adverse events related to the procedure (i.e. alloimmunization) was reported. During the follow-up neither typical acute complications of SCD, such as acute chest syndrome, splenic sequestration, stroke, bone necrosis, priapism, nor long term complications like renal failure, cerebrovascular or retinic damage, pseudoxanthoma like manifestations were observed. None of the typical iron overload consequences (hypogonadism, growth failure, hypotiroidism and diabetes) were noted. Concerning cardiac function, all subjects showed a left ventricular ejection fraction &gt; 60% with no evidence of pulmonary hypertension, evaluated by echocardiography. LIC, assessed by SQUID or liver biopsy, was normal in all patients except one. This patient developed iron overload, requiring steady iron chelation therapy, due to CRT before entering the MEEX program at the age of 26 yrs. Cardiac T2* measured by MRI resulted &gt;22mms in each patient. During the observation period 5 hospitalizations (4 acute cholecystitis and 1 Venous Occlusive Crisis) occurred. Only one patient needed chronic analgesic therapy to relieve head femur necrosis pain developed before starting periodic MEEXes. According to age all patients attended school or had a regular job. Discussion: The long-term follow-up revealed the above described procedure to be safe and efficacious in preventing acute and chronic complications of SCD in patients unresponsive to HU therapy, allowing them to have a good quality of life. This approach is less invasive and significantly less expensive than both CTR-chelation and automated EEX. Furthermore since MEEX is feasible and easy to manage it should be considered a treatment option also in developing countries, according to ASH SDC Policy Statements.

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