A case report of recurrent primary posterior mediastinal perivascular epithelioid cell tumour compressing the right inferior pulmonary vein, atria, and inferior vena cava.

Background: Perivascular epithelioid cell tumours (PEComas) are rare soft tissue neoplasms that commonly occur in the uterus, skin, and liver and less commonly in the retroperitoneum, colon, and mediastinum. Case summary: A 36-year-old male patient with a history of mediastinal PEComa status post resection, essential hypertension, and atrial fibrillation status post appendage ligation currently not on anticoagulation presented with a 1-week history of fevers, chills, productive cough, chest pain, dyspnoea on exertion, loss of appetite, and general weakness. Vital signs, physical exam, laboratory data, electrocardiogram, and chest radiograph were grossly unremarkable. A multimodality imaging approach utilizing transthoracic echocardiogram, transoesophageal echocardiogram (TEE), cardiac magnetic resonance imaging (cMRI), and computed tomography angiography of the chest, abdomen, and pelvis revealed a local 40 mm × 53 mm globular bilobed vascularized scar-free posterior mediastinal mass arising from the roof of the left and right atria and extending superiorly to the main pulmonary artery and inferiorly to the inferior vena cava. Based on the mass' size and proximity to vital structures and tumour recurrence, the case was presented during tumour board rounds, and the outcome was to surgically resect the mass and then have the patient follow up with medical oncology and radiation oncology for possible chemotherapy and radiation, respectively. Discussion: Perivascular epithelioid cell tumours are rare, and mediastinal PEComas are even rarer, warranting a multimodality imaging approach involving TEE and cMRI and a multidisciplinary approach involving anaesthesiologists, cardiologists, cardiothoracic surgeons, medical oncologists, pathologists, radiologists, and radiation oncologists.

Generating OCT B-Scan DME images using optimized Generative Adversarial Networks (GANs).

Diabetic Macular Edema (DME) represents a significant visual impairment among individuals with diabetes, leading to a dramatic reduction in visual acuity and potentially resulting in irreversible vision loss. Optical Coherence Tomography (OCT), a technique that produces high-resolution retinal images, plays a vital role in the clinical assessment of this condition. Physicians typically rely on OCT B-Scan images to evaluate DME severity. However, manual interpretation of these images is susceptible to errors, which can lead to detrimental consequences, such as misdiagnosis and improper treatment strategies. Hence, there is a critical need for more reliable diagnostic methods. This study aims to address this gap by proposing an automated model based on Generative Adversarial Networks (GANs) to generate OCT B-Scan images of DME. The model synthesizes images from patients' baseline OCT B-Scan images, which could potentially enhance the robustness of DME detection systems. We employ five distinct GANs in this study: Deep Convolutional GAN, Conditional GAN, CycleGAN, StyleGAN2, and StyleGAN3, drawing comparisons across their performance. Subsequently, the hyperparameters of the best-performing GAN are fine-tuned using Particle Swarm Optimization (PSO) to produce more realistic OCT images. This comparative analysis not only serves to improve the detection of DME severity using OCT images but also provides insights into the appropriate choice of GANs for the effective generation of realistic OCT images from the baseline OCT datasets.

Fuzzy Logic-Based System for Identifying the Severity of Diabetic Macular Edema from OCT B-Scan Images Using DRIL, HRF, and Cystoids.

Diabetic Macular Edema (DME) is a severe ocular complication commonly found in patients with diabetes. The condition can precipitate a significant drop in VA and, in extreme cases, may result in irreversible vision loss. Optical Coherence Tomography (OCT), a technique that yields high-resolution retinal images, is often employed by clinicians to assess the extent of DME in patients. However, the manual interpretation of OCT B-scan images for DME identification and severity grading can be error-prone, with false negatives potentially resulting in serious repercussions. In this paper, we investigate an Artificial Intelligence (AI) driven system that offers an end-to-end automated model, designed to accurately determine DME severity using OCT B-Scan images. This model operates by extracting specific biomarkers such as Disorganization of Retinal Inner Layers (DRIL), Hyper Reflective Foci (HRF), and cystoids from the OCT image, which are then utilized to ascertain DME severity. The rules guiding the fuzzy logic engine are derived from contemporary research in the field of DME and its association with various biomarkers evident in the OCT image. The proposed model demonstrates high efficacy, identifying images with DRIL with 93.3% accuracy and successfully segmenting HRF and cystoids from OCT images with dice similarity coefficients of 91.30% and 95.07% respectively. This study presents a comprehensive system capable of accurately grading DME severity using OCT B-scan images, serving as a potentially invaluable tool in the clinical assessment and treatment of DME.

A case report of successful transaneurysmal repair of a giant isolated membranous ventricular septal aneurysm.

Background: Isolated membranous ventricular septal aneurysms are infrequent in clinical practice. Furthermore, current guidelines do not dictate how to diagnose or manage such lesions. Case summary: A 54-year-old male patient with a history of essential hypertension and tobacco use presented with chest pain associated with dyspnoea and nausea. Electrocardiogram was unrevealing. Physical exam was significant for a diastolic murmur heard best in the apex. Computed tomography angiography of the chest revealed an aneurysm measuring 5 cm in diameter along the ascending aorta. Transoesophageal echocardiography showed that the aneurysm originated from the membranous ventricular septum, coursed along the ascending aorta, and ended anteriorly to the surface of the right ventricle and ascending aorta. Cardiac magnetic resonance imaging confirmed these findings and demonstrated that the aneurysm comprised of two loculations. Given the size of the aneurysm and its proximal location to major cardiovascular structures, percutaneous repair was considered unsafe. Following a multidisciplinary meeting, the lesion was successfully resected via a transaneurysmal approach. Discussion: Isolated membranous ventricular septal aneurysms are best imaged via a combination of transoesophageal echocardiogram and cardiac magnetic resonance imaging and best managed via a multidisciplinary approach for optimal outcomes.

A case report of percutaneous MitraClip implantation in an adult with a double-outlet right ventricle.

Background: Atrioventricular valve regurgitation (AVVR) is present in up to 75% of Fontan patients, and it is associated with an increased risk of Fontan circulation failure, morbidity, and mortality. Traditional treatment options include surgical repair vs. surgical replacement. We present, to the best of our knowledge, one of the first cases of successful trans-catheter repair of severe common AVVR using the MitraClip device. Case summary: A 20-year-old male with a history of double-outlet right ventricle (DORV) with unbalanced common atrioventricular canal to the right ventricle, severely hypoplastic left ventricle, and total anomalous pulmonary venous return status post-Fontan procedure presented with progressively worsening dyspnoea on exertion. Transoesophageal echocardiogram demonstrated severe common AVVR. After discussion of the case during the adult congenital heart disease multidisciplinary conference, patient underwent successful placement of two MitraClip devices, reducing the regurgitation from torrential to moderate. Discussion: MitraClip therapy can be used to alleviate symptoms in patients deemed as high risk for surgery. However, careful attention must be paid to haemodynamics before and after clip placement, which may predict short-term clinical outcomes.

Cardiac patterns for differentiation of neurogenic stunned myocardium in aneurysmal subarachnoid hemorrhage versus acute ischemic stroke.

AIMS: Neurogenic stunned myocardium (NSM) has heterogeneous presentations for acute ischemic stroke (AIS) and aneurysmal subarachnoid hemorrhage (SAH). We sought to better define NSM and differences between AIS and SAH by evaluating individual left ventricular (LV) functional patterns by speckle tracking echocardiography (STE). METHODS: We evaluated consecutive patients with SAH and AIS. Via STE, LV longitudinal strain (LS) values of basal, mid, and apical segments were averaged and compared. Different multivariable logistic regression models were created by defining stroke subtype (SAH or AIS) and functional outcome as dependent variables. RESULTS: One hundred thirty-four patients with SAH and AIS were identified. Univariable analyses using the chi-squared test and independent samples t-test identified demographic variables and global and regional LS segments with significant differences. In multivariable logistic regression analysis, when comparing AIS to SAH, AIS was associated with older age (OR 1.07, 95% CI 1.02-1.13, p = 0.01), poor clinical condition on admission (OR 7.74, 95% CI 2.33-25.71, p < 0.001), decreased likelihood of elevated admission serum troponin (OR .09, 95% CI .02-.35, p < 0.001), and worse LS basal segments (OR 1.18, 95% CI 1.02-1.37, p = 0.03). CONCLUSION: In patients with neurogenic stunned myocardium, significantly impaired LV contraction by LS basal segments was found in patients with AIS but not with SAH. Individual LV segments in our combined SAH and AIS population were also not associated with clinical outcomes. Our findings suggest that strain echocardiography may identify subtle forms of NSM and help differentiate the NSM pathophysiology in SAH and AIS.

Mortality and Complications of COVID-19 Among Adult Congenital Heart Disease Patients: A Retrospective Cohort Study Using the National Inpatient Sample Database.

This study examines in-hospital mortality and complicated COVID-19 infection among adult congenital heart disease (ACHD) patients admitted with COVID-19, using the National Inpatient Sample (NIS). A total of 4219 COVID-19 patients with ACHD were included. We demonstrated that COVID-19 patients with ACHD were more likely to experience in-hospital mortality (OR 1.04, 95% CI 1.04-1.04, P < 0.01) and complicated COVID-19 infection (OR: 1.30, 95% CI: 1.11-1.53, P < 0.01). In our sub-group analysis, COVID-19 patients with tetralogy of Fallot (TOF) had higher mortality and COVID-19 patients with atrial septal defects (ASD) had a higher incidence of complicated infection when compared to COVID-19 patients with all other ACHDs. Risk factors for mortality among COVID-19 patients with ACHD include advanced age, lower income, unrepaired ACHD, malnutrition, and chronic liver disease. Accordingly, we recommend aggressive preventive care with vaccination and non-pharmacologic measures in order to improve survival for ACHD patients.

Noninvasive Assessment With Transthoracic Echocardiography in End-Stage Heart Failure.

Heart failure is a common cardiac condition that carries a substantial risk of morbidity and mortality despite advances in management. Echocardiography plays a central role in its diagnosis, elucidation of mechanisms, and detailed hemodynamic analysis. In this E-Challenge, the authors review a few transthoracic echocardiographic findings that yield insights into the hemodynamics.

Population Risk Factors for Severe Disease and Mortality in COVID-19 in the United States during the Pre-Vaccine Era: A Retrospective Cohort Study of National Inpatient Sample.

Background-Previous studies on coronavirus disease 2019 (COVID-19) were limited to specific geographical locations and small sample sizes. Therefore, we used the National Inpatient Sample (NIS) 2020 database to determine the risk factors for severe outcomes and mortality in COVID-19. Methods-We included adult patients with COVID-19. Univariate and multivariate logistic regression was performed to determine the predictors of severe outcomes and mortality in COVID-19. Results-1,608,980 (95% CI 1,570,803-1,647,156) hospitalizations with COVID-19 were included. Severe complications occurred in 78.3% of COVID-19 acute respiratory distress syndrome (ARDS) and 25% of COVID-19 pneumonia patients. The mortality rate for COVID-19 ARDS was 54% and for COVID-19 pneumonia was 16.6%. On multivariate analysis, age > 65 years, male sex, government insurance or no insurance, residence in low-income areas, non-white races, stroke, chronic kidney disease, heart failure, malnutrition, primary immunodeficiency, long-term steroid/immunomodulatory use, complicated diabetes mellitus, and liver disease were associated with COVID-19 related complications and mortality. Cardiac arrest, septic shock, and intubation had the highest odds of mortality. Conclusions-Socioeconomic disparities and medical comorbidities were significant determinants of mortality in the US in the pre-vaccine era. Therefore, aggressive vaccination of high-risk patients and healthcare policies to address socioeconomic disparities are necessary to reduce death rates in future pandemics.

Management of Recurrent Myocarditis Due to Desmoplakin Cardiomyopathy: Diagnostic and Therapeutic Challenges.

We present the case of a 25-year-old woman with desmoplakin cardiomyopathy-related myocarditis. Her high-sensitivity troponin and symptoms improved with pulse steroid therapy and mycophenolate mofetil. The literature lacks data to effectively guide the management of recurrent myocarditis in desmoplakin cardiomyopathy. (Level of Difficulty: Advanced.).

Fractional Flow Reserve in End-Stage Liver Disease.

Fractional flow reserve (FFR) determines the functional significance of epicardial stenoses assuming negligible venous pressure (Pv) and microvascular resistance. However, these assumptions may be invalid in end-stage liver disease (ESLD) because of fluctuating Pv and vasodilation. Accordingly, all patients with ESLD who underwent right-sided cardiac catheterization and coronary angiography with FFR as part of their orthotopic liver transplantation evaluation between 2013 and 2018 were included in the present study. Resting mean distal coronary pressure (Pd)/mean aortic pressure (Pa), FFR, and Pv were measured. FFR accounting for Pv (FFR - Pv) was defined as (Pd - Pv)/(Pa - Pv). The hyperemic effect of adenosine was defined as resting Pd/Pa - FFR. The primary outcome was all-cause mortality at 1 year. In 42 patients with ESLD, 49 stenoses were interrogated by FFR (90% were <70% diameter stenosis). Overall, the median model for ESLD score was 16.5 (10.8 to 25.5), FFR was 0.87 (0.81 to 0.94), Pv was 8 mm Hg (4 to 14), FFR-Pv was 0.86 (0.80 to 0.94), and hyperemic effect of adenosine was 0.06 (0.02 to 0.08). FFR-Pv led to the reclassification of 1 stenosis as functionally significant. There was no significant correlation between the median model for ESLD score and the hyperemic effect of adenosine (R = 0.10). At 1 year, 13 patients had died (92% noncardiac in etiology), and patients with FFR ≤0.80 had significantly higher all-cause mortality (73% vs 17%, p = 0.001. In conclusion, in patients with ESLD who underwent orthotopic liver transplantation evaluation, Pv has minimal impact on FFR, and the hyperemic effect of adenosine is preserved. Furthermore, even in patients with the predominantly angiographically-intermediate disease, FFR ≤0.80 was an independent predictor of all-cause mortality.

Novel ensemble of optimized CNN and dynamic selection techniques for accurate Covid-19 screening using chest CT images.

The world is significantly affected by infectious coronavirus disease (covid-19). Timely prognosis and treatment are important to control the spread of this infection. Unreliable screening systems and limited number of clinical facilities are the major hurdles in controlling the spread of covid-19. Nowadays, many automated detection systems based on deep learning techniques using computed tomography (CT) images have been proposed to detect covid-19. However, these systems have the following drawbacks: (i) limited data problem poses a major hindrance to train the deep neural network model to provide accurate diagnosis, (ii) random choice of hyperparameters of Convolutional Neural Network (CNN) significantly affects the classification performance, since the hyperparameters have to be application dependent and, (iii) the generalization ability using CNN classification is usually not validated. To address the aforementioned issues, we propose two models: (i) based on a transfer learning approach, and (ii) using novel strategy to optimize the CNN hyperparameters using Whale optimization-based BAT algorithm + AdaBoost classifier built using dynamic ensemble selection techniques. According to our second method depending on the characteristics of test sample, the classifier is chosen, thereby reducing the risk of overfitting and simultaneously produced promising results. Our proposed methodologies are developed using 746 CT images. Our method obtained a sensitivity, specificity, accuracy, F-1 score, and precision of 0.98, 0.97, 0.98, 0.98, and 0.98, respectively with five-fold cross-validation strategy. Our developed prototype is ready to be tested with huge chest CT images database before its real-world application.

Pericardiocentesis induced right ventricular changes in patients with and without pulmonary hypertension.

BACKGROUND: Pericardial effusion drainage in patients with significant pulmonary hypertension (PH) has been questioned because of hemodynamic collapse concern, mainly because of right ventricular (RV) function challenging assessment. We aimed to assess RV function changes related to pericardiocentesis in patients with and without PH. METHODS: Consecutive patients with symptomatic moderate-to-large pericardial effusion who had either echocardiographic or clinical signs of cardiac tamponade and who underwent pericardiocentesis from 2013 to 2018 were included. RV speckle-tracking echocardiography analysis was performed before and after pericardiocentesis. Patients were stratified by significant PH (pulmonary artery systolic pressure [PASP] ≥50 mm Hg). RESULTS: The study cohort consisted of 76 patients, 23 (30%) with PH. In patients with PH, both end-diastolic and end-systolic areas (EDA, ESA) increased significantly after pericardiocentesis (22.6 ± 8.0 cm2 -26.4 ± 8.4 cm2 , P = .01) and (15.9 ± 6.3 cm2 -18.7 ± 6.5 cm2 , P = .02), respectively. However, RV function indices including fractional area change (FAC: 30.6 ± 13.7%-29.1 ± 8.8%, P = .61) and free-wall longitudinal strain (FWLS: -16.7 ± 6.7 to -15.9 ± 5.0, P = .50) remained unchanged postpericardiocentesis. In contrast, in the non-PH group, after pericardiocentesis, EDA increased significantly (20.4 ± 6.2-22.4 ± 5.9 cm2 , P = .006) but ESA did not (14.9 ± 5.7 vs 15.0 ± 4.6 cm2 , P = .89), and RV function indices improved (FAC 27.9 ± 11.7%-33.1 ± 8.5%, P = .003; FWLS -13.6 ± 5.4 to -17.2 ± 3.9%, P < .001). CONCLUSION: Quantification of RV size and function can improve understanding of echocardiographic and hemodynamic changes postpericardiocentesis, which has the potential to guide management of PH patients with large pericardial effusion.

Three-Dimensional Echocardiographic Left Atrial Appendage Volumetric Analysis.

BACKGROUND: Left atrial appendage (LAA) echocardiographic assessment is difficult because of the complex shape and relatively small size of the LAA. Three-dimensional (3D) echocardiographic imaging can overcome the limitations of two-dimensional imaging. Pulsed-wave Doppler is the only currently standard LAA functional parameter. The aim of this study was to test a new approach for 3D echocardiographic volumetric analysis to obtain LAA ejection fraction (EF), its size and shape. METHODS: Transesophageal two-dimensional and 3D LAA images were prospectively obtained in 159 consecutive patients. LAA volumes were measured from 3D echocardiographic images using available software. Pulsed-wave Doppler was considered the reference value for LAA function and was used for comparison with LAA EF. Comparison with cardiac computed tomography was performed in a subgroup of 32 patients. Comparisons included linear regression and Bland-Altman analyses. Repeated measurements were performed to assess measurement variability. RESULTS: Nine patients were excluded because of suboptimal image quality (94% feasibility). Three-dimensional LAA calculated EF was in good agreement with LAA pulsed-wave measurements. Three-dimensional morphologic evaluation showed that 43% of the patients had "chicken wing," 33% "cactus," 19% "windsock," and 5% cauliflower shapes. At the time of data acquisition, patients with atrial fibrillation had nonsignificantly larger LAA end-systolic and end-diastolic volumes, leading to lower calculated EFs. Three-dimensional echocardiographic LAA end-systolic volumes were in good agreement with cardiac computed tomography (r = 0.75), with small biases (mean, -2.5 ± 3.9 ml). Reproducibility was better for larger LAA volumes. CONCLUSIONS: A novel 3D echocardiographic approach can determine the geometry, size, and function of the LAA. A new parameter, LAA EF, provides functional quantitation.

Pooled Analysis of PFO Occluder Device Trials in Patients With PFO and Migraine.

BACKGROUND: Although observational studies have shown percutaneous patent foramen ovale (PFO) closure to be a safe means of reducing the frequency and duration of migraine, randomized clinical trials have not met their primary efficacy endpoints. OBJECTIVES: The authors report the results of a pooled analysis of individual participant data from the 2 randomized trials using the Amplatzer PFO Occluder to assess the efficacy and safety of percutaneous device closure as a therapy for episodic migraine with or without aura. METHODS: The authors analyzed individual patient-level data from 2 randomized migraine trials (the PRIMA [Percutaneous Closure of Patent Foramen Ovale in Migraine With Aura] and PREMIUM [Prospective Randomized Investigation to Evaluate Incidence of Headache Reduction in Subjects with Migraine and PFO Using the Amplatzer PFO Occluder Compared to Medical Management] studies). Efficacy endpoints were mean reduction in monthly migraine days, responder rate (defined as ≥50% reduction in monthly migraine attacks), mean reduction in monthly migraine attacks, and percentage of patients who experienced complete cessation of migraine. The safety endpoint was major procedure- and device-related adverse events. RESULTS: Among 337 subjects, 176 were randomized by blocks to device closure and 161 to medical treatment only. At 12-month follow-up, the analysis met 3 of the 4 efficacy endpoints: mean reduction of monthly migraine days (-3.1 days vs. -1.9 days; p = 0.02), mean reduction of monthly migraine attacks (-2.0 vs. -1.4; p = 0.01), and number of subjects who experienced complete cessation of migraine (14 [9%] vs. 1 [0.7%]; p < 0.001). For the safety analysis, 9 procedure-related and 4 device-related adverse events occurred in 245 subjects who eventually received devices. All events were transient and resolved. CONCLUSIONS: This pooled analysis of patient-level data demonstrates that PFO closure was safe and significantly reduced the mean number of monthly migraine days and monthly migraine attacks, and resulted in a greater number of subjects who experienced complete migraine cessation.

Comparison of Contractility Patterns on Left Ventriculogram Versus Longitudinal Strain by Echocardiography in Patients With Takotsubo Cardiomyopathy.

BACKGROUND: Takotsubo cardiomyopathy (TTC) is characterized by transient left ventricular (LV) dysfunction, electrocardiographic changes that can mimic acute myocardial infarction (MI), and release of myocardial enzymes in the absence of obstructive coronary artery disease (CAD). Conventionally, gross visual assessment of LV angiogram has been used to classify TTC. We aim to compare quantitative assessment of different regions of LV on angiogram and segmental strain on transthoracic echo to determine a better way to classify TTC rather than conventional qualitative visual assessment. METHODS: We conducted a retrospective observational study of 20 patients diagnosed with TTC who had LV angiogram and transthoracic echocardiograms performed on presentation that were suitable for analysis. Twenty LV angiograms were analyzed using Rubo DICOM viewer software. Areas of different LV regions were measured in diastole and systole, and percentage changes in area of these regions were calculated. Percentage changes in area of less than 10% was considered "akinetic." On the other hand, using echocardiograms of these patients, LV regional longitudinal strain (LS) was derived from speckle-tracking analysis. These findings were compared to determine concordance between both modalities. RESULTS: On quantitative analysis of 20 LV angiograms, the area of all the three LV regional (apex, mid ventricle, and base) shortening (>10%) was observed in 16 patients (80%) during systole as compared to diastole. However, only 4 out of 20 patients (20%) were noted to have apical region area change of <10% between diastole and systole. Analysis of LV regional LS patterns of 20 patients showed that 14 patients had abnormal values (> -18%) in all three LV regions: apex, mid ventricle, and base. The apical region was the most severely affected region (mean LS -13.9%), followed by the basal region (mean -14.7%) and the mid ventricular region (mean -15.1%). Comparing the results of both modalities showed that there was 35% (n = 7) concordance in the results noted for base and apical regions of the LV, whereas only 20% (n = 4) concordance was noted in mid ventricular region. CONCLUSION: Contractility (shortening) on LV angiogram is present in a majority of patients in the three LV regions, but contractility assessed by LS is impaired in most of them. The concordance in both quantitative assessment modalities was low. LV angiogram may not be an accurate imaging modality to assess contractility patterns in Takotsubo patients, and echocardiographic LS analysis should be taken as the preferred imaging modality.

Clonal Hematopoiesis of Indeterminate Potential and Cardiovascular Disease.

PURPOSE OF REVIEW: The purpose of this review article is to summarize the preclinical and clinical evidence supporting the notion of clonal hematopoiesis of indeterminate potential (CHIP), highlight current knowledge gap, and provide future directions. RECENT FINDINGS: Epidemiological studies show that advanced age is a major risk factor for the development of cardiovascular disease (CVD) and cancer, the two leading causes of morbidity and mortality worldwide. While the negative effect of aging on CVD is a reflection of cumulative exposure to various established traditional CVD risk factors, genetic sequencing of whole blood-derived DNA recently revealed that clonal mutations in myeloid stem cells are associated with higher risks of cardiovascular events and hematopoietic malignancies. The clinical repercussions of this biological state, termed CHIP, are increasingly appreciated. Historically, CHIP has been associated with an increased risk of hematological malignancies. However, new research is showing that CHIP is also associated with an increased risk of several cardiac-related conditions, including atherosclerosis, myocardial infarction, aortic valve stenosis, and congestive heart failure. CHIP is increasingly being appreciated worldwide as a CVD risk factor, and further studies are needed to better understand the complex relationship between these two disorders.