Speckle tracking echocardiography and ß-thalassemia major. A systematic review.

Heart disease is among the primary causes of morbidity and mortality in ß-thalassemia major (ß-TM). Conventional echocardiography has failed to identify myocardial dysfunction at an early stage among these patients, thus speckle tracking echocardiography (STE) has been lately used. The objectives of this review were to 1) identify all published studies having evaluated myocardial strain among ß-TM patients, 2) gather their results, 3) compare their findings and 4) propose recommendations based on these data. Literature search was conducted in PubMed, SCOPUS and Cohrane Library. Data regarding left ventricular global longitudinal (LV-GLS), circumferential (LV-GCS) and radial strain (LV-GRS), right ventricular longitudinal strain (RV-GLS), left and right atrial strain were extracted. Thirty-five studies (34 original articles and 1 meta-analysis) have met the inclusion criteria. LV-GLS has been reported being worse in patients compared to controls in 13 of 21 studies, LV-GCS in 7 of 11 studies, LV-GRS in 6 of 7 studies, RV-GLS in 2 of 3 studies and left atrial strain in all case-control studies. Myocardial iron overload (MIO) patient subgroups had worse LV-GLS in 6 of 15 studies, LV-GCS in 2 of 7 studies and LV-GRS in none of 7 studies. A small number of studies suggest left atrial strain correlation with electrical atrial ectopy and atrial fibrillation. It is suggested that STE should be applied supplementary to conventional echocardiography for early identification of myocardial dysfunction among ß-TM patients. Potential myocardial strain utilities could be screening for myocardial iron overload, left ventricular diastolic dysfunction and atrial fibrillation.

Left atrial deformation indices in ß-thalassemia major patients.

The presence of atrial cardiomyopathy in ß-thalassemia major (ß-TM) patients complicates their clinical condition. The diagnosis is challenging even with cardiac magnetic resonance (CMR) imaging. Novel echocardiographic techniques are applied to increase the diagnostic yield. Fifty-six ß-TM patients and thirty age and sex-matched controls were included in the present cross-sectional study. Heart rate, PR duration, and P axis were measured by electrocardiography, left ventricular ejection fraction (LVEF) and end-diastolic diameter (LVEDD), ratio between early mitral inflow velocity and mitral annular early diastolic velocity (E/e'), left atrial volume index (LAVI), left atrial strain at reservoir (LASr), conduit (LAScd) and contraction (LASct) phases respectively, left ventricular global longitudinal strain (GLS) by echocardiography, and T2* calculation in patient group by CMR. PR duration, LVEF, LAVI, E/e', GLS, and left atrial deformation parameters differed between patients and controls (p <0.05). In patient group, left atrial strain was correlated with PR duration, LAVI, E/e', GLS, and T2* (p <0.05). T2* was correlated only with left atrial deformation indices (p <0.05). Patients with a history of atrial fibrillation were older, had lower heart rate, prolonged PR, increased E/e' and LAVI, and impaired left atrial strain (p <0.05). LASct differed relative to the presence of atrial fibrillation and myocardial iron overload. Atrial strain could be of clinical use in the early detection of atrial cardiomyopathy. An impaired LASct could identify ß-TM patients with undetected episodes of atrial fibrillation. Finally, left atrial strain may be helpful in myocardial iron load estimation.

Transthoracic ultrasound; seeking the heart in a patient with a large left-sided cirrhotic hydrothorax.

A case of a patient with liver cirrhosis and a large left-sided pleural effusion displacing the heart rightward is presented and the best views to acquire images enabling evaluation of the cardiac function are highlighted. Understanding the modified intrathoracic anatomy in patients with pleural effusions enables quick and focused assessment and can shorten evaluation time while preserving high image quality.

Stroke and presence of patent foramen ovale in sickle cell disease.

Sickle cell disease (SCD) is an inherited monogenic hemoglobinopathy characterized by formation of sickle erythrocytes under conditions of deoxygenation. Sickle erythrocytes can lead to thrombus formation and vaso-occlusive episodes that may result in hemolytic anemia, pain crisis and multiple organ damage. Moreover, SCD is characterized by endothelial damage, increased inflammatory response, platelet activation and aggravation, and activation of both the intrinsic and the extrinsic coagulation pathways. Cerebrovascular events constitute an important clinical complication of SCD. Children with SCD have a 300-fold higher risk of acute stroke and by the age of 45 about 25% of patients have suffered an overt stoke. Management and prevention of stroke in patients with SCD is not well defined. Moreover, the presence of patent foramen ovale (PFO) increases the risk of the occurrence of an embolic cerebrovascular event. The role of PFO closure and antiplatelet or anticoagulation therapy has not been well investigated. Moreover, during COVID-19 pandemic and taking into account the increased rates of thrombotic events and the difficulties in blood transfusion, management of SCD patients is even more challenging and difficult, since data are scarce regarding stroke occurrence and management in this specific population in the COVID-19 era. This review focuses on pathophysiology of stroke in patients with SCD and possible treatment strategies in the presence of PFO.

Acute Chest Syndrome in Sickle Cell Disease: Clinical Presentation and Outcomes. The Experience of a Single Thalassemia and Sickle Cell Unit in a University Hospital.

Acute chest syndrome (ACS) is a common cause of death for sickle cell disease patients. This syndrome is defined as: respiratory symptoms, new X-ray findings developed and/or fever; ACS requires prompt treatment to avoid clinical deterioration and death in adults with sickle cell disease. Sixteen episodes of acute chest syndrome were studied in 16 adults with sickle cell disease. The clinical and radiological findings, treatment, response and outcome of the episode were evaluated respectively. The patient's past history and comorbidities were taken into account in the outcome and days of hospitalization. Fourteen patients recovered with no sequelae; one patient who required mechanical ventilation also recovered; one patient died due to pulmonary emboli. The mean hospitalization days were 7.43.

Severity of heart failure and health-related quality of life in beta-thalassemia patients: a cross-sectional study.

Cardiovascular complications account for a substantial increase in morbidity and mortality in beta-thalassemia patients. Many patients have structural heart disease, and some of them present with symptomatic heart failure (HF). Quality of life (QOL) of beta-thalassemia patients is lower than that of the general population. The aim of our study was to explore the relationship between HF stages and QOL in beta-thalassemia patients. Seventy-three consecutive adult beta-thalassemia patients took part in this cross-sectional study. Stages of HF, classified with increasing severity as A, B, and C, were determined based on ACC/AHA guidelines. QOL was assessed using the SF-36 questionnaire. Fifteen patients had stage C HF, twenty-eight had stage B HF, and the remaining were considered stage A patients, as beta thalassemia is a predisposing factor for HF. All QOL domains except for bodily pain were significantly lower in stage C patients than in stage A patients. Stage C patients had significantly lower QOL scores for physical functioning, role physical, and social functioning domains than stage B patients. Stage B patients' QOL differed from stage A patients only in the vitality domain. In the multiple regression analysis which took several demographic and clinical factors into account, stage of HF was the most important factor associated with QOL, and negatively and significantly related to five QOL domains, namely physical functioning, role physical, general health, social functioning, and vitality. In conclusion, QOL is negatively affected by the severity of heart failure in beta-thalassemia patients.

The Contemporary Role of Speckle Tracking Echocardiography in Cirrhotic Cardiomyopathy.

Cirrhotic cardiomyopathy (CCM) is characterized by elevated cardiac output at rest, an inability to further increase contractility under stress, and diastolic dysfunction. The diagnosis of CCM is crucial as it can lead to complications during liver transplantation. However, its recognition poses challenges with conventional echocardiography techniques. Speckle tracking echocardiography (STE), particularly global longitudinal strain (GLS), is a novel index that enhances the diagnostic efficacy of echocardiography for both ischemic and non-ischemic cardiomyopathies. GLS proves more sensitive in identifying early systolic dysfunction and is also influenced by advanced diastolic dysfunction. Consequently, there is an expanding scope for GLS utilization in cirrhotic cases, with newly updated diagnostic criteria for CCM incorporating GLS. Specifically, systolic dysfunction is now defined as either a left ventricular ejection fraction below 50% or an absolute GLS below 18%. However, conflicting data on GLS alterations in liver cirrhosis patients persist, as many individuals with advanced disease and a poor prognosis exhibit a hyperdynamic state with preserved or increased GLS. Consequently, the presence of CCM, according to the updated criteria, does not exhibit a significant association-in the majority of studies-with the severity of liver disease and prognosis. Furthermore, information on other indices measured with STE, such as left atrial and right ventricular strain, is promising but currently limited. This review aims to offer a critical assessment of the existing evidence concerning the application of STE in patients with liver cirrhosis.

Resting strain analysis to identify myocardial ischemia in patients with advanced chronic kidney disease.

BACKGROUND: Chronic kidney disease (CKD) is associated with higher incidence of cardiovascular death. Screening for coronary artery disease in asymptomatic or mildly symptomatic patients is challenging. OBJECTIVE: The aim of this study was to investigate the incremental value of resting deformation analysis in predicting positive results for myocardial ischemia during stress transthoracic echocardiography in patients with end-stage CKD. METHODS: Sixty-one patients (mean age: 62.3 ± 11.8, 65.7% men) with end-stage CKD were included in the study. Patients underwent a resting transthoracic echocardiogram and a dobutamine stress contrast echo (DSE) protocol. Positive results of DSE were defined as stress-induced left ventricular (LV) wall motion abnormalities. RESULTS: The study cohort had normal or mildly impaired systolic function: mean LV ejection fraction (EF) was 49.2% (±10.4) and mean LV global longitudinal strain (GLS) was 14.4% (±4.5). Half of our population had impaired left atrial (LA) strain: mean LA reservoir, conduit, and contractile reserve were 24.1% (±12.6), 10.6% (±5.9), and 13.6% (±9.2), respectively. DSE was positive for ischemia in 55.7%. A significant negative association with DSE results was found for LV EF, LV GLS and the conduit phase of LA strain. Both LV and LA dimensions showed positive correlation with presence of ischemia in DSE. Multivariate logistic regression analysis showed that LV GLS was independently associated with DSE (p = 0.007), after controlling for covariates, with high diagnostic accuracy. CONCLUSION: Resting LV deformation could predict positive results during DSE, thus may be useful to better identify renal patients who might benefit from coronary artery screening.

Left Atrium: A New Prognostic Marker and Therapeutic Target in Secondary Mitral Regurgitation?

Mitral regurgitation (MR) is one of the most common valvular abnormalities worldwide and can be subdivided into primary and secondary causes. Atrial MR consists of a novel type of secondary MR (SMR), most often observed in patients with AF and heart failure with preserved ejection fraction. The main pathophysiological mechanism of atrial MR is mitral valve annular dilatation. Recently published studies have highlighted the clinical significance of left atrium (LA) evaluation in the early diagnosis and prognosis of patients with primary MR. However, there are little data regarding the role of the LA in SMR. The present literature review aims to elucidate the use of the echocardiographic parameters regarding LA evaluation in the prognosis prediction and therapeutic strategy of patients with SMR.

HFA-PEFF score as an independent predictor of 2-year mortality in liver cirrhosis patients.

BACKGROUND AND AIMS: Cardiovascular complications of liver cirrhosis include high cardiac output heart failure with reduced afterload and diastolic dysfunction. Heart Failure Association Pre-test assessment, Echocardiography and natriuretic peptide, Functional testing, Final etiology (HFA-PEFF) (values 0-6) is a diagnostic tool for heart failure. It is a cluster of echocardiographic and biochemical parameters. The purpose of this prospective study was to assess if HFA-PEFF score is associated with the severity of liver disease and to determine its prognostic significance in patients with cirrhosis. METHODS: Patients with cirrhosis without an acute event were enrolled. A comprehensive echocardiography study was conducted, brain natriuretic peptide and N-terminal-pro-B-type Natriuretic Peptide levels were measured and HFA-PEFF score was calculated. All patients were prospectively followed up after enrollment. The study end-point was defined as the composite of all-cause death. RESULTS: Seventy-two consecutive patients [median age 59 years (inter-quartile range: 52-65), 72.2% males, Model for End-stage Liver Disease (MELD) 12.9 (8.5-16.3)] were included. The 52.8% had a high HFA-PEFF score (5 or 6) and the 47.2% intermediate/low score (0-4). HFA-PEFF score was correlated with MELD score (rho = 0.566, P < 0.001), but not with age, SBP and heart rate. The median follow-up was 24.2 (9.5-38.8) months. The 1- and 2-year cumulative mortality was higher in patients with high HFA-PEFF compared to those with intermediate/low (log-rank P < 0.001 for both). The predicted 2-year probability of survival in high vs intermediate/low HFA-PEFF was found to be a prognostic factor of death even when adjusted for age, gender and MELD score [hazard ratio = 3.539 (1.134-11.046), P = 0.029]. CONCLUSION: HFA-PEFF score is high among patients with liver cirrhosis, especially those with advanced liver disease. HFA-PEFF score is associated with survival even when adjusting for cirrhosis severity.

The Fundamental Role of Stress Echo in Evaluating Coronary Artery Disease in Specific Patient Populations.

Stress echocardiography (SE) was initially used for assessing patients with known or suspected coronary heart disease by detecting and evaluating myocardial ischemia and viability. The implementation of SE has gradually been extended to several cardiovascular diseases beyond coronary artery disease, and SE protocols have been modified and adapted for the detection of coronary artery disease (CAD) or other cardiovascular diseases in specific patient populations. This review attempts to summarize current data concerning SE implementation and clinical value in these specific and diverse populations: patients with an intramural course of a coronary artery, known as a myocardial bridge, chronic severe or end-stage hepatic disease, chronic severe or end-stage kidney disease, cardiac allograft vasculopathy, patients scheduled for solid-organ transplantation and other intermediate and high-risk surgery and, finally, patients treated with anticancer drugs or radiotherapy.

Twelve-lead and signal-averaged electrocardiographic parameters among beta-thalassemia major patients.

BACKGROUND: The majority of beta thalassemia major (ß-TM) patients suffer from cardiac disease, while a significant proportion of them die suddenly. Twelve-lead and signal-averaged electrocardiography (SAECG) 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: A total of 47 ß-TM patients and 30 healthy controls were submitted to 12-lead and signal-averaged electrocardiography. Basic electrocardiographic parameters and prevalence of LPs were recorded. Basic echocardiographic parameters were estimated by transthoracic echocardiography. T2* was calculated by cardiac magnetic resonance imaging wherever available. RESULTS: ß-TM patients demonstrated a more prolonged PR interval (167.74 msec vs 147.07 msec) (P = .043), a higher prevalence of PR prolongation (21.05% vs 0%) (P = .013), and a higher prevalence of LPs (18/47, 38.3% vs 2/30, 6.7%) (P = .002) compared with controls. The prevalence of atrial fibrillation among b-TM patients was estimated at 10.64%. Patients had also greater E/e' ratio (8.35, SD = 2.2 vs 7, SD = 2.07) (P = .012) and LAVI (30.7 mL/m2, SD = 8.76 vs 24.6 mL/m2, SD = 6.57) (P = .002) than controls. Regression analysis showed that QTc and LAVI could correctly predict the presence of LPs in the 80.9% of the patients. CONCLUSIONS: ß-TM patients have a higher prevalence of a prolonged PR interval, 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.