scholarly journals Left ventricular dysfunction by strain echocardiography in thalassemia patients: a pilot study

Author(s):  
Akshay Ashok Bafna ◽  
Hetan C. Shah

Background: To evaluate the myocardial function and its correlation with serum ferritin and the number of transfusions in beta-thalassemia major patients by using standard echocardiography and left ventricular strain imaging.Methods: This was a cross-sectional exploration study comprised of 56 beta-thalassemia patients conducted at a tertiary-care center in India between September 2016 and August 2017. Patients with age less than 18 years, diagnosed with thalassemia major, recipients of >20 units of blood transfusions, and normal Left Ventricular (LV) function by 2D-echocardiography were included in the study. Severity of iron overload was determined by using serum ferritin levels and LV strain imaging parameters were evaluated by using strain values of 17 LV segments.Results: A total of 56 beta-thalassemia patients were included in the study. Of these, 29(51.8%) patients were boys and 27(48.2%) patients were girls with a mean age of 7.8±1.84 years. Average serum ferritin level was found to be 4089.83 ng/dl. Strain values of the basal lateral wall of the left ventricle were significantly abnormal in patients who received more (>80) transfusions compared with those who received lesser transfusions (p=0.025 and p=0.045), respectively. Patients with serum ferritin >6000 ng/ml had impaired strain (p=0.03).Conclusions: Conventional echocardiographic parameters and Left Ventricular Ejection Fraction (LVEF) do not provide adequate information about LV dysfunction. Systolic strain index imaging of the LV indicated the presence of early LV systolic dysfunction in patients who received a greater number of blood transfusions and patients with higher serum ferritin levels.

Hematology ◽  
2009 ◽  
Vol 2009 (1) ◽  
pp. 664-672 ◽  
Author(s):  
Heather A. Leitch ◽  
Linda M. Vickars

AbstractThe myelodysplastic syndromes (MDS) are characterized by cytopenias and risk of transformation to acute myeloid leukemia (AML). Although new treatments are available, a mainstay in MDS remains supportive care, which aims to minimize the impact of cytopenias and transfusion of blood products. Red blood cell (RBC) transfusions place patients at risk of iron overload (IOL). In beta-thalassemia major (BTM), IOL from chronic RBC transfusions inevitably leads to organ dysfunction and death. With iron chelation therapy (ICT), survival in BTM improved from the second decade to near normal and correlated with ICT compliance. Effects of ICT in BTM include reversal of cardiac arrhythmias, improvement in left ventricular ejection fraction, arrest of hepatic fibrosis, and reduction of glucose intolerance.It is not clear whether these specific outcomes are applicable to MDS. Although retrospective, recent studies in MDS suggest an adverse effect of transfusion dependence and IOL on survival and AML transformation, and that lowering iron minimizes this impact. These data raise important points that warrant further study. ICT is potentially toxic and cumbersome, is costly, and in MDS patients should be initiated only after weighing potential risks against benefits until further data are available to better justify its use. Since most MDS patients eventually require RBC transfusions, the public health implications both of transfusion dependence and ICT in MDS are considerable. This paper summarizes the impact of cytopenias in MDS and treatment approaches to minimize their impact, with a focus on RBC transfusions and their complications, particularly with respect to iron overload.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
D Patsourakos ◽  
C Aggeli ◽  
K Gatzoulis ◽  
S Delicou ◽  
Y Dimitroglou ◽  
...  

Abstract Background/Introduction The majority of beta thalassemia major (β-TM) patients suffer from cardiac disorders, while a significant proportion of them die suddenly. Twelve-lead and signal-averaged electrocardiography are simple, inexpensive, readily available tools for identifying an unfavorable arrhythmiological substrate by detecting the presence of arrhythmias, conduction abnormalities and late potentials (LPs) in these patients. Methods 47 β-TM patients and 30 healthy controls were submitted to twelve-lead and signal-averaged electrocardiography. Basal rhythm, heart rate, PR interval duration, QRS complex duration and morphology, QTc interval duration and prevalence of LPs were recorded. Results β-TM patients demonstrated a more prolonged PR segment (167.74 msec vs. 147.07 msec) (p=0.043), a higher prevalence of PR prolongation (21.05% vs. 0%) (p=0.013) and a higher prevalence of LPs (18/47, 38.3% vs. 2/30, 6.7%) (p=0.002) compared with controls. In particular, every single SAECG parameter significantly differed among patients compared with controls. Among patients, left ventricular ejection fraction was marginally lower and QTc more prolonged among LPs positive subgroup compared with LPs negative subgroup. The prevalence of atrial fibrillation among b-TM patients was estimated at 10.64%. Conclusions β-TM patients have a higher prevalence of a prolonged PR segment, atrial fibrillation and LPs. Twelve-lead and SAECG performance was feasible in all subjects and constitutes a readily available tool for assessing myocardial electrophysiological alterations in this patient group, that could have significant impact on survival and quality of life with timely application of appropriate treatment. Funding Acknowledgement Type of funding source: None


Tomography ◽  
2021 ◽  
Vol 7 (2) ◽  
pp. 130-138
Author(s):  
Narumol Chaosuwannakit ◽  
Pattarapong Makarawate ◽  
Chinnadol Wanitpongpun

Objective: Cardiac T2* magnetic resonance imaging (MRI) has recently attracted considerable attention as a non-invasive method for detecting iron overload in various organs in thalassemia major patients. This study aimed to identify the prevalence of cardiac siderosis in thalassemia major patients and evaluate cardiac T2* MRI for monitoring cardiac siderosis before and after patients receive iron chelation therapy and its relation to serum ferritin, left ventricular ejection fraction, and liver iron concentration. The information gathered would be used for the direct monitoring, detection, and treatment of complications early on. Methods: A total of 119 thalassemia major patients were recruited in the present study. The cardiac T2* MRI was compared to serum ferritin levels, liver iron concentration (LIC), and left ventricular ejection fraction. All patients were classified into four groups based on their cardiac siderosis as having normal, marginal, mild to moderate, or severe cardiac iron overload. At the follow-up at years one, three, and five, the cardiac T2* MRI, LIC, serum ferritin, and left ventricular ejection fraction (LVEF) were determined. Results: The prevalence of cardiac siderosis with cardiac T2* MRI ≤ 25 ms was 17.6% (n = 21). There was no correlation between cardiac T2* MRI and serum ferritin, liver iron concentration, and LVEF (p = 0.39, 0.54, and 0.09, respectively). During one year to five years’ follow-up periods, cardiac T2* MRI (ms) in patients with severe cardiac siderosis had significantly improved from 8.5 ± 1.49 at baseline to 33.9 ± 1.9 at five years (p < 0.0001). Patients with severe, mild-moderate, marginal, and no cardiac siderosis had median LIC (mg/g dw) of 23.9 ± 6.5, 21.6 ± 13.3, 25.3 ± 7.7, and 19.9 ± 5.5 at baseline, respectively. Conclusions: This study supports the use of cardiac T2* MRI to monitor cardiac iron overload in patients who have had multiple blood transfusions. Early diagnosis and treatment of patients at risk of cardiac siderosis is a reasonable method of reducing the substantial cardiac mortality burden associated with myocardial siderosis. Cardiac T2* MRI is the best test that can identify at-risk patients who can be managed with optimization of their chelation therapy.


Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 3886-3886
Author(s):  
Renzo Galanello ◽  
Antonio Piga ◽  
Maria- Eliana Lai ◽  
Gianluca Forni ◽  
Fabrice Danjou ◽  
...  

Abstract Introduction: Cardiac iron overload secondary to red blood cell transfusion is a common complication of thalassemia major despite the wide use of chelation therapy and cardiac disease still account for up to 70% of deaths in these patients. Furthermore, myocardial siderosis can be accurately assessed by cardiovascular magnetic resonance (CMR) using T2* sequence. Available chelators seem to have unique profiles of organ iron removal. We report a retrospective analysis of the effect of three available chelators on cardiac iron in patients with thalassemia major. Methods: Fifty-two patients on subcutaneous desferrioxamine (DFO), 28 on deferiprone (DFP) and 28 on deferasirox (DFX) are included in the study. DFO was administered at a mean dosage of 36 ± 8 mg/kg/d for 10 to 14 hours per day for 21 ± 13 months, DFP at 86 ± 10 mg/kg/d for 23 ± 12 months and DFX at 27 ± 6mg/kg/d for 15 ± 5 months. T2* was measured according to Westwood et al (2005). Entry criteria included in each patient a value of T2* lower than 20 ms at baseline or at final assessment. Left ventricular ejection fraction (LVEF) was measured by echocardiography. Results: At baseline, the 3 treatment groups did not show any significant difference in age blood consumption and cardiac T2* (DFO group: 13.4 ± 4.5 ms, DFP 13.9 ± 3.7 and DFX 12.8 ± 3.7; p=0.573).. At the last evaluation mean cardiac T2* was slightly increased in patients on DFO (13.9 ± 8.6 ms, p=0.8) and in those on DFX (13.8 ± 4.4 ms, p=0.04), while was substantially increased in patients on DFP (21.7 ± 9.2 ms, p=0.001). To correct for the different duration of treatment we calculated the percentage monthly cardiac T2* changes that were significantly higher in patients on DFP (1,84 ± 1.94), as compared to patients on DFO (0.2 3 ± 2.15) or DFX (0.45 ± 1.49) (p<0.001). No differences were detected between mean LVEF at baseline and at last assessment in all 3 groups. Conclusions: In this retrospective study monotherapy with deferiprone was significantly more effective than desferrioxamine and deferasirox in alleviating myocardial siderosis in patients with beta-thalassemia major. Further studies are needed to understand if the cardiac T2* changes are influenced by the chelator dosages.


2021 ◽  
Vol 10 (Supplement_1) ◽  
Author(s):  
E Marcusohn ◽  
O Kobo ◽  
M Postnikov ◽  
D Epstein ◽  
Y Agmon ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: None. Background  The diagnosis of atrial fibrillation (AF) induced cardiomyopathy can be challenging. It relies on ruling out other causes of dilated cardiomyopathy, upon recovery of left ventricular ejection fraction (LVEF) following return to sinus rhythm (SR). Aim  The aim of this study was to identify clinical and echocardiographic predictors for developing new dilated cardiomyopathy in patients with AF or atrial flutter (AFL). Methods  This is a retrospective study conducted in a large tertiary care center. Patients that suffered deterioration of LVEF under 50% during AF demonstrated by pre-cardioversion trans-esophageal echocardiography (TEE) were compared to those with preserved LV function during AF. All patients had documented preserved LVEF at baseline (EF &gt;50%) while in SR. Patients with a previous history of reduced LVEF during SR were excluded. Results From a total of 482 patients included in the final analysis, 80 (17%) patients had reduced LV function and 402 (83%) had preserved LV function during the pre-cardioversion TEE. Patients with reduced LVEF were more likely to be male and with a more rapid ventricular response during AF/AFL. A history of prosthetic valves was also identified as a risk factor for reduced LVEF. Patients with reduced LVEF also had higher incidence of TR and RV dysfunction. Conclusion In "real world" experience, male patients with rapid ventricular response during AF or AFL are more prone to LVEF reduction. Patients with prosthetic valves are also at risk for LVEF reduction during AF/AFL. Lastly, TR and RV dysfunction may indicate relatively long-standing AF with an associated reduction in LVEF.


Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Marcus Sandri ◽  
Stephan Gielen ◽  
Norman Mangner ◽  
Volker Adams ◽  
Sandra Erbs ◽  
...  

Background: The concept of ventricular-arterial coupling implies that LV-function is determined by the three factors left ventricular diastolic, left ventricular systolic and arterial elastance. We have previously documented an improvement in endothelial function and systolic LV-function in patients with chronic heart failure (CHF) after 6 months of exercise training (ET). It remains, however, unclear, how shorter ET periods may affect endothelial, systolic and diastolic ventricular function as echocardiographic parameters related to ventricular arterial coupling in patients with CHF. METHODS: In this ongoing study we randomised 43 patients with stable CHF (age 60.3 ± 2.9 years, EF 27.4 ± 1.7%, VO 2 max 14.7 ± 4.3ml/kg*min) to a training or a control group (C). Patients in the training group exercised 4 times daily at 70% of the individual heart rate reserve for 4 weeks under supervision. At baseline and after 4 weeks the E/A ratio and septal/lateral E’/A’ velocities were determined by echocardiography with tissue Doppler. Exercise capacity was measured by ergospirometry and flow-mediated dilatation (FMD) was assessed by high-resolution radial ultrasound. RESULTS: After only 4 weeks of ET oxygen uptake at peak exercise increased from 14.9 ± 3.3 to 18.1 ± 4.7 ml/min/kg, (p<0.01 vs. C) in training subjects. Left ventricular ejection fraction improved from 26.8 ± 4.6 to 33.1 ± 5.5% (p<0.05 vs. C) in patients of the training group while it remained unchanged in the control group. E/A-ratio mended from 0.63 ± 0.12 to 0.81 ± 0.22 (p<0.01 vs. C) in training patients. Septal E’ velocities increased from 5.5 ± 0.5 to 7.8 ± 1.4 cm/s in training patients (p<0.05 vs. C). FMD of the radial artery improved from 8.2 ± 2.1 to 15.2 ± 3.8% (p<0.01 vs. C) as a result of ET. CONCLUSIONS: Only 4 weeks of endurance training are highly effective with significantly improved FMD accompanied by an emended systolic and diastolic LV-function. We hypothesise that the improvement in LV-EF in training patients may be caused by a corrected ventricular-arterial coupling: ventricular diastolic relaxation and effective endothelial function are ameliorated resulting in an augmentation of stroke volume.


Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Daniela Ravizzoni Dartora ◽  
Adrien Flahualt ◽  
Carolina Nobre Pontes ◽  
Gabriel Altit ◽  
Alyson Deprez ◽  
...  

Introduction: Preterm (PT) birth is associated with increased risk of cardiovascular diseases (CVD) and heart failure. We previously reported left ventricular (LV) mitochondrial dysfunction in a rat model mimicking the deleterious conditions associated with PT birth. Whether mitochondrial function is altered in humans born PT and associated with LV function changes is unknown. We aimed to determine if serum humanin levels, a mitochondrial-derived peptide with cytoprotective effects, are altered in humans born PT and are associated with impaired myocardial function. Methods: Data were obtained from 55 young adults born PT (<30 weeks of gestational age, GA) compared to 54 full-term (T) controls of the same age. Serum humanin levels were determined by ELISA and LV ejection fraction (LVEF) by echocardiography. Results are shown as median (interquartile range) and comparisons between groups were performed using non-parametric tests. Results: Individuals were evaluated at 23.3 (21.4, 25.3) years, and age and sex distribution were similar between groups. Median GA was 27.5 (26.2, 28.4) weeks in the PT group. Humanin levels (pg/ml) were 132.9 (105.1, 189.3) and 161.1 (123.6, 252) in the PT and the T groups, respectively (p=0.0414). LVEF was within the normal range and similar between groups. Lower LVEF was associated with lower humanin levels (p<0.001), and this association was observed both in the term (p=0.002) and the preterm (p=0.047) groups. Conclusions: Serum humanin levels are lower in adult born PT. Since lower humanin levels are also associated with lower LVEF, our results suggest that mitochondrial alterations could play a role in the long-term adverse cardiovascular consequences of PT birth. Humanin analogs improve LV function in experimental models. Our results pave the way for future studies exploring humanin as a therapeutic avenue for the prevention and treatment of CVD in individuals born PT.


Author(s):  
Debojyoti Bhattacharjee ◽  
Jayati Roy Choudhury ◽  
Kasturi Mukherjee ◽  
Kheya Mukherjee

Introduction: Dengue, a vector borne viral infection transmitted by Aedes mosquito has recently become a major public health concern in the tropical regions of the world. In addition to the two major life threatening complications- Dengue Haemorrhagic Fever (DHF) and Dengue Shock Syndrome (DSS), a variety of cardiac complications have been recognised, the most common being myocarditis. Aim: To study and compare the quantity of different cardiac biomarkers in patients of Dengue Fever with and without myocarditis. Materials and Methods: This was a hospital-based retrospective observational study done in a Tertiary Care Hospital, Kolkata, West Bengal, India from June 2019 to November 2019. Dengue patients with diagnosed myocarditis on day 7 of fever based on electro and echocardiogram changes of left ventricular ejection fraction less than 50% were considered as cases (n=41). Age and sex matched dengue patients with normal electro and echocardiogram changes were considered as control (n=43). After obtaining Institutional Ethics Committee Clearance, laboratory data were collected from samples coded and assayed for markers of acute cardiac myocyte damage such as total Creatine Kinase (CK), CK-Muscle Brain (CK-MB), Troponin T (Trop T) and cardiac failure biomarker N-Terminal pro Brain Natriuretic Peptide (NT-proBNP). Statistical analysis of the data was performed using Statistical Package for Social Sciences (SPSS 20). Results: Cardiac biomarkers CK, CK-MB, Trop T and NT-proBNP levels in cases were higher compared to controls (p-value <0.05). Trop T and NT-proBNP were positively correlated to each other (r-value: 0.44). Trop T changes could also predict significantly the rise in NT-proBNP in circulation (p<0.05). Conclusion: It reconfirmed the need of routine monitoring of cardiac biomarkers in conjunction with other cardiac function tests in early diagnosis and or management of myocarditis, a severe complication of Dengue Viral Infection (DENV).


2018 ◽  
Vol 2018 ◽  
pp. 1-5 ◽  
Author(s):  
Suraj Haridas Upadya ◽  
M. S. Rukmini ◽  
Sowmya Sundararajan ◽  
B. Shantharam Baliga ◽  
Nutan Kamath

Background. Thalassemia is the most common genetic disorder worldwide. Use of iron chelators has improved survival but endocrine complications have become more frequent. The frequency of hypothyroidism in Beta Thalassemia Major (BTM) children ranges from 6 to 30 %. Thyroid dysfunction mainly occurs by gland infiltration, chronic tissue hypoxia, free radical injury, and organ siderosis. Objectives. (a) To evaluate the thyroid function status in chronically transfused children with BTM, in the first and second decade of life and (b) to study the influence of factors like duration and amount of blood transfusions, serum ferritin level, and iron chelation therapy on thyroid function. Methodology. BTM children, 3 years old and above, on regular blood transfusions with serum ferritin > 1500 mcg/l were included in the study. Thyroid function and ferritin assessment was done using ELISA kits. Autoimmune thyroiditis was ruled out by antithyroid peroxidase and antithyroglobulin antibody testing. Results. A study population of 83 children consisted of 49 boys (59%) and 34 girls (41%). 4.8% of the children had evidence of subclinical hypothyroidism. Among them two belonged to the first decade and the other two to the second decade of life. Mean TSH, FT4, and ferritin values among children with thyroid dysfunction were 6.38 ± 0.83 mIU/ml, 1.08 ± 0.45 ng/dl, and 3983.0±1698.30 ng/ml, respectively. The severity of thyroid dysfunction was statistically significantly associated with higher serum TSH values in children in the second decade of life with a p value = 0.001. No other significant correlation was found between oral chelation, amount and duration of blood transfusion, or serum ferritin levels. Conclusion. Subclinical hypothyroidism was the thyroid dysfunction observed in our study. Regular blood transfusions with adequate chelation may decrease incidence of thyroid dysfunction.


Circulation ◽  
2000 ◽  
Vol 102 (suppl_3) ◽  
Author(s):  
Bruno Pouzet ◽  
Jean-Thomas Vilquin ◽  
Albert A. Hagège ◽  
Marcio Scorsin ◽  
Emmanuel Messas ◽  
...  

Background —Autologous skeletal myoblast (SM) transplantation improves function of infarcted myocardium, but pretransplantation cultures remain a complex process. This study assessed whether it could be optimized by muscle preconditioning with the local anesthetic bupivacaine or even bypassed with the use of the so-called mince technique. Methods and Results —Muscle preconditioning consisted of intramuscular injections of the tibialis anterior of rats, 2 days before harvest. After 7 days of culture, the number of available myoblasts was significantly increased compared with nonconditioned controls (1 683 147 versus 85 300, P =0.0013). The mince technique was then assessed. A myocardial infarction was created in 66 rats by coronary artery ligation. One week later, rats were reoperated on and intramyocardially injected with culture medium alone (controls, n=23), autologous cultured SM (3.5×10 6 , n=21), or autologous muscle minced into a fine slurry, which was immediately transplanted (n=22). All muscles had been preconditioned. Left ventricular function was assessed by 2D echocardiography. Whereas end-diastolic volumes expanded over time in all groups, left ventricular ejection fraction (%, mean±SEM) was increased only in the cultured SM–transplanted group at 1 ( P =0.0006) and 2 months ( P =0.0008) versus baseline (37.52±1.92 and 40.92±2.17 versus 30.34±1.74), with a significant additional benefit between 1 and 2 months ( P =0.0069). Conclusions —Cell culture remains mandatory for SM transplantation to be successful but, in a clinical perspective, this process can be made more expeditious by preharvest muscle conditioning with bupivacaine, which greatly enhances the baseline cell yield.


Sign in / Sign up

Export Citation Format

Share Document