Left ventricular systolic and diastolic functions in patients with sickle cell anemia

Şan, Mustafa; Demirtaş, Mustafa; Burgut, Refik; Birand, Ahmet; Başlamişh, Fikri

doi:10.1007/bf01617389

Cited by 28 publications

(8 citation statements)

References 13 publications

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“…Cardiac alterations in SCD have been previously studied. In agreement with published literature, we found in our SCD patients a preserved LV ejection fraction 5,19,8,11,13 along with LV dilation [5][6][7]4,8,10,11,13 and an increase in LV mass [5][6][7]14,4,8,10,11,13 . Such LV enlargement has been hypothesized to reflect a remodeling mechanism to adapt to the elevation in stroke volume induced by the chronic anemia-related decrease in blood oxygenation in such disease to match metabolic demand 29 .…”

Section: Discussionsupporting

confidence: 92%

“…Left ventricular (LV) alterations in SCD have been widely described, including LV dilation [4][5][6][7][8][9][10][11][12][13] , an increase in LV mass 5,7,14,4,8,[10][11][12][13] , as well as diastolic dysfunction 7,11,13,15 . Today, up to 25% of SCD deaths are related to cardiovascular causes 16 and diastolic dysfunction is a known independent risk factor for mortality in SCD patients 17 .…”

Section: Introductionmentioning

confidence: 99%

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Abnormalities in aortic properties: a potential link between left ventricular diastolic function and ventricular—aortic coupling in sickle cell disease

Bollache

Kachenoura

Lang

et al. 2016

Int J Cardiovasc Imaging

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Left ventricular (LV) diastolic dysfunction in patients with sickle cell disease (SCD) is associated with increased mortality. However, its mechanisms are not well known, preventing the development of effective therapies. We hypothesized that patients with SCD have altered aortic properties despite normal blood pressure, which may contribute towards the development of diastolic dysfunction. We studied 31 stable adult patients with SCD (32 ± 7 years) and 12 healthy controls of similar age (29 ± 10 years) who underwent echocardiography and cardiovascular magnetic resonance (CMR) imaging on the same day. Echocardiographic measurements of mitral inflow and mitral annulus velocities were used to evaluate LV diastolic function. CMR imaging included standard LV function evaluation and myocardial tissue characterization as well as velocity-encoded images of the ascending aorta to measure aortic diastolic cross-sectional area, distensibility, as well as peaks and volumes of the global, forward and backward blood flow rate. Compared to controls, SCD patients had increased aortic diastolic area, global stroke volume, and both forward and backward flow, while aortic distensibility and peripheral blood pressure were similar. Furthermore, peak backward flow rate and volume were able to discriminate between patients with and without diastolic dysfunction. Our findings show that some aortic properties are altered in SCD patients and may be associated with diastolic dysfunction despite normal systolic blood pressure. If confirmed in larger studies, these aortic changes could be a novel therapeutic target to prevent or delay the development of LV diastolic dysfunction in SCD and thus potentially improve outcomes in these patients.

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Section: Discussionsupporting

confidence: 92%

Section: Introductionmentioning

confidence: 99%

Abnormalities in aortic properties: a potential link between left ventricular diastolic function and ventricular—aortic coupling in sickle cell disease

Bollache

Kachenoura

Lang

et al. 2016

Int J Cardiovasc Imaging

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“…Recently, in SCD children with no history of overt stroke or seizures, we have reported that higher systolic blood pressure (SBP) is associated with increased risk of an silent cerebral infarction (SCI) [13], a common form of neurological injury among children with SCD, occurring in at least 27% prior to six years of life and 37% by 14 years of life [14,15]. In addition, previous clinical studies have shown that the SCD may have a deleterious effect on myocardium, which contributes to abnormal rates of change in left ventricular cavity size and systolic/diastolic function in SCD patients [16-18]. Indeed, nearly one third of adults with SCD also develop an elevated tricuspid regurgitant velocity (TRV) that is associated with a much higher death rate in SCD patients compared to patients with SCD without elevated TRV.…”

Section: Introductionmentioning

confidence: 99%

Genome-Wide Meta-Analysis of Systolic Blood Pressure in Children with Sickle Cell Disease

et al. 2013

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In pediatric sickle cell disease (SCD) patients, it has been reported that higher systolic blood pressure (SBP) is associated with increased risk of a silent cerebral infarction (SCI). SCI is a major cause of neurologic morbidity in children with SCD, and blood pressure is a potential modulator of clinical manifestations of SCD; however, the risk factors underlying these complications are not well characterized. The aim of this study was to identify genetic variants that influence SBP in an African American population in the setting of SCD, and explore the use of SBP as an endo-phenotype for SCI. We conducted a genome-wide meta-analysis for SBP using two SCD cohorts, as well as a candidate screen based on published SBP loci. A total of 1,617 patients were analyzed, and while no SNP reached genome-wide significance (P-value<5.0x10-8), a number of suggestive candidate loci were identified. The most significant SNP, rs7952106 (P-value=8.57x10-7), was in the DRD2 locus on chromosome 11. In a gene-based association analysis, MIR4301 (micro-RNA4301), which resides in an intron of DRD2, was the most significant gene (P-value=5.2x10-5). Examining 27 of the previously reported SBP associated SNPs, 4 SNPs were nominally significant. A genetic risk score was constructed to assess the aggregated genetic effect of the published SBP variants, demonstrating a significant association (P=0.05). In addition, we also assessed whether these variants are associated with SCI, validating the use of SBP as an endo-phenotype for SCI. Three SNPs were nominally associated, and only rs2357790 (5’ CACNB2) was significant for both SBP and SCI. None of these SNPs retained significance after Bonferroni correction. Taken together, our results suggest the importance of DRD2 genetic variation in the modulation of SBP, and extend the aggregated importance of previously reported SNPs in the modulation of SBP in an African American cohort, more specifically in children with SCD.

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“…Cardiovascular causes account for nearly one‐fourth of all premature deaths in adults with sickle cell anemia (SCA) 1–3 . The maladaptive cardiac remodeling seen in SCA underlies the cardio‐pathology of SCA and worsens with age 4–7 . Moderate–severe anemia from the ongoing hemolysis results in a compensatory hemodynamic state comprising increased cardiac output, pan‐chamber dilation, and left ventricular (LV) hypertrophy, seen in both children and adults with SCA 8–11 .…”

Section: Introductionmentioning

confidence: 99%

“…[1][2][3] The maladaptive cardiac remodeling seen in SCA underlies the cardio-pathology of SCA and worsens with age. [4][5][6][7] Moderate-severe anemia from the ongoing hemolysis results in a compensatory hemodynamic state comprising increased cardiac output, pan-chamber dilation, and left ventricular (LV) hypertrophy, seen in both children and adults with SCA. [8][9][10][11] LV systolic dysfunction is a rare complication in adults.…”

mentioning

confidence: 99%

Longitudinal effect of disease‐modifying therapy on left ventricular diastolic function in children with sickle cell anemia

et al. 2023

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Cardiac abnormalities seen in sickle cell anemia (SCA) include diastolic dysfunction, which has been shown to be associated with high morbidity and early mortality. The effect of disease‐modifying therapies (DMT) on diastolic dysfunction is poorly understood. We prospectively evaluated the effects of hydroxyurea and monthly erythrocyte transfusions on diastolic function parameters over 2 years. A total of 204 subjects with HbSS or HbSβ0‐thalassemia (mean age 11 ± 3.7 years), unselected for disease severity, had diastolic function assessed with surveillance echocardiograms twice, 2 years apart. During this 2‐year observation period, 112 participants received DMTs (hydroxyurea, n = 72, monthly erythrocyte transfusions, n = 40), 34 initiated hydroxyurea, and 58 did not receive any DMT. The entire cohort showed an increase in left atrial volume index (LAVi) of 3.40 ± 10.86 mL/m2, p = .001 over 2 years. This increase in LAVi was independently associated with anemia, high baseline E/e′ or LV dilation. Individuals not exposed to DMT were younger (mean age 8.8 ± 2.9 years), but at baseline their prevalence of abnormal diastolic parameters was similar to that of the DMT‐exposed participants who were older (mean age 12 ± 3.8 years). Participants on DMTs saw no improvement in diastolic function over the study period. In fact, participants on hydroxyurea saw a possible worsening in diastolic parameters (14% increase in LAVi and ~5% decrease in septal e′) but also a ~9% decrease in fetal hemoglobin (HbF) levels. Further studies are needed to evaluate if exposure to DMT for a longer duration or achieving higher HbF might be beneficial in alleviating diastolic dysfunction.

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Left ventricular systolic and diastolic functions in patients with sickle cell anemia

Cited by 28 publications

References 13 publications

Abnormalities in aortic properties: a potential link between left ventricular diastolic function and ventricular—aortic coupling in sickle cell disease

Abnormalities in aortic properties: a potential link between left ventricular diastolic function and ventricular—aortic coupling in sickle cell disease

Genome-Wide Meta-Analysis of Systolic Blood Pressure in Children with Sickle Cell Disease

Longitudinal effect of disease‐modifying therapy on left ventricular diastolic function in children with sickle cell anemia

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