Valve-in-Valve Transcatheter Mitral Valve Replacement in a Very High-Risk Octagenerian Patient: A Case Report.

Degeneration of the surgical bioprosthetic valves remains one of the most common complications of surgical valve replacement. Redo surgery is the gold standard, but unfortunately, most of these patients are deemed inoperable because of the high perioperative mortality. Transcatheter implantation of a new valve inside the degenerated bioprosthesis (valve-in-valve (ViV)) has emerged as an alternative solution. A 79-year-old patient with a medical history of surgical replacement of the mitral valve with a bioprosthetic valve, coronary artery bypass graft surgery (CABG) with implantation of the left internal mammary artery (LIMA) to the left anterior descending artery (LAD), paroxysmal atrial fibrillation, and chronic kidney disease was referred to our hospital for ViV transcatheter mitral valve replacement (TMVR). He had recent hospitalizations with pulmonary edema caused by severe stenosis of the bioprosthetic valve and his perioperative mortality for a redo surgery was very high (EuroSCORE II: 13.72%). The ViV TMVR was performed with a transseptal approach and after the implantation of the new valve, the mean pressure gradient was dropped from 19.39 to 2.33 mmHg. The procedure was technically successful and the patient was discharged asymptomatic.

[Factors associated with left ventricular diastolic dysfunction in adults from a region of the Andes in Peru]. / Factores asociados con la disfunción diastólica del ventrículo izquierdo en adultos de una región de los Andes en Perú.

Objective: To determine the factors associated with left ventricular diastolic dysfunction (LVDD) in adults residing in a region of the Andes in Peru. Method: A case-control study was conducted on adults living at an altitude of more than 3000 meters in Peru. Cases consisted of patients diagnosed with LVDD through echocardiography, whereas controls were adults without LVDD, as confirmed by echocardiography. Results: A total of 50 cases and 100 controls were included in the study. Among them, 38.7% had high blood pressure, and 41.3% were overweight. Upon adjusted analysis, age 60 or older (aOR: 4.06; 95%CI: 1.29-12.8), female sex (aOR: 2.24; 95%CI: 1.01-4.96) and left ventricular hypertrophy (aOR: 3.17; 95%CI: 1.41-7.17) were identified as statistically significant factors associated with LVDD. Conclusions: The risk of LVDD is associated with older adults, female gender, and left ventricular hypertrophy among individuals residing above 3000 meters altitude in a region of the Andes, in Peru.

Objetivo: Determinar los factores asociados con la disfunción diastólica del ventrículo izquierdo (DDVI) en adultos de una región de los Andes, en Perú. Método: Estudio de casos y controles en adultos residentes a más de 3000 metros de altitud en Perú. Los casos fueron pacientes adultos diagnosticados con DDVI por ecocardiografía, y los controles fueron adultos sin DDVI por ecocardiografía. Resultados: Se incluyeron 50 casos y 100 controles. El 38.7% tuvieron hipertensión arterial y el 41.3% sobrepeso. En el análisis ajustado, la edad de 60 o más años (ORa: 4.06; IC95%: 1.29-12.8), el sexo femenino (ORa: 2.24; IC95%: 1.01-4.96) y la hipertrofia ventricular izquierda (ORa: 3.17; IC95%: 1.41-7.17) fueron factores estadísticamente significativos. Conclusiones: El riesgo de DDVI estuvo asociado a los adultos mayores, las mujeres y los pacientes con hipertrofia ventricular izquierda que viven por encima de los 3000 metros de altitud en una región de los Andes, en Perú.

Conotruncal Heart Defects: A Narrative Review of Molecular Genetics, Genomics Research and Innovation.

Congenital heart defects (CHDs) are most prevalent cardiac defects that occur at birth, leading to significant neonatal mortality and morbidity, especially in the developing nations. Among the CHDs, conotruncal heart defects (CTDs) are particularly noteworthy, comprising a significant portion of congenital cardiac anomalies. While advances in imaging and surgical techniques have improved the diagnosis, prognosis, and management of CTDs, their molecular genetics and genomic substrates remain incompletely understood. This expert review covers the recent advances from January 2016 onward and examines the complexities surrounding the genetic etiologies, prevalence, embryology, diagnosis, and clinical management of CTDs. We also emphasize the known copy number variants and single nucleotide variants associated with CTDs, along with the current planetary health research efforts aimed at CTDs in large cohort studies. In all, this comprehensive narrative review of molecular genetics and genomics research and innovation on CTDs draws from and highlights selected works from around the world and offers new ideas for advances in CTD diagnosis, precision medicine interventions, and accurate assessment of prognosis and recurrence risks.

Cardiovascular diseases as risk factors of post-COVID syndrome: a systematic review.

BACKGROUND: A growing proportion of people experience incomplete recovery months after contracting coronavirus disease 2019 (COVID-19). These COVID-19 survivors develop a condition known as post-COVID syndrome (PCS), where COVID-19 symptoms persist for > 12 weeks after acute infection. Limited studies have investigated PCS risk factors that notably include pre-existing cardiovascular diseases (CVD), which should be examined considering the most recent PCS data. This review aims to identify CVD as a risk factor for PCS development in COVID-19 survivors. METHODS: Following the Preferred Reporting Items for Systematic Review and Meta-Analyses (PRISMA) checklist, systematic literature searches were performed in the PubMed, Scopus, and Web of Science databases from the earliest date available to June 2023. Data from observational studies in English that described the association between CVD and PCS in adults (≥ 18 years old) were included. A minimum of two authors independently performed the screening, study selection, data extraction, data synthesis, and quality assessment (Newcastle-Ottawa Scale). The protocol of this review was registered under PROSPERO (ID: CRD42023440834). RESULTS: In total, 594 studies were screened after duplicates and non-original articles had been removed. Of the 11 included studies, CVD including hypertension (six studies), heart failure (three studies), and others (two studies) were significantly associated with PCS development with different factors considered. The included studies were of moderate to high methodological quality. CONCLUSION: Our review highlighted that COVID-19 survivors with pre-existing CVD have a significantly greater risk of developing PCS symptomology than survivors without pre-existing CVD. As heart failure, hypertension and other CVD are associated with a higher risk of developing PCS, comprehensive screening and thorough examinations are essential to minimise the impact of PCS and improve patients' disease progression.

Association of increased participation in social activity in later life with risk of all-cause mortality and heart diseases in older people: results from the Chinese Longitudinal Healthy Longevity Survey (CLHLS).

Background: Previous studies have shown social activity is associated with reduced risk of health outcomes. However, among older people (≥65 years) who were socially inactive at baseline, limited study explored whether increased participation in social activity in later life was associated with reduced risk of health outcomes; therefore, using the data from the Chinese Longitudinal Healthy Longevity Survey, the study was performed. Methods: The study outcomes were 10-year all-cause mortality (sample number = 9,984) and 10-year heart diseases (sample number = 7,496). The exposure was the change of social activity frequency. Cox regression analysis was used for data analysis. Results: During the follow-up, there were 6,407 all-cause mortalities and 1,035 heart diseases, respectively. Kaplan-Meier analysis demonstrated that cumulative incidences of all-cause mortality were significantly lower in participants with changes into more frequent social activity (log-rank p < 0.001), while no significant difference was observed for heart diseases (log-rank p = 0.330). Compared with the subgroup who never participated in social activity at baseline, adjusted HRs of all-cause mortality were 0.79 (95% CI: 0.70-0.90, p < 0.001), 0.78 (95% CI: 0.63-0.96, p = 0.019), 0.74 (0.59-0.92, p = 0.006), and 0.70 (95% CI: 0.56-0.88, p = 0.002) for the subgroup of switching to sometimes, the subgroup of switching to once a month, the subgroup of switching to once a week, and the subgroup of switching to everyday, respectively. The corresponding HRs of heart diseases were 0.83 (95% CI: 0.65-1.08, p = 0.170), 0.82 (95% CI: 0.51-1.31, p = 0.412), 0.91 (0.58-1.42, p = 0.675) and 0.75 (95% CI: 0.47-1.20, p = 0.227), respectively. Stratified and sensitivity analyses revealed similar results. Conclusion: Among older people who never participated in social activity, increased participation in social activity in later life was associated with reduced risk of all-cause mortality, but was not associated with reduced risk of heart diseases.

Differential Deep RNA Sequencing for Diagnostic Detection of Microbial Infections in Inflammatory Cardiomyopathy.

BACKGROUND: Inflammatory heart disease can be triggered by a variety of causes, both infectious and noninfectious in nature. We hypothesized that inflammatory cardiomyopathy is potentially related to microbial infection. METHODS: In this retrospective study, we used deep RNA sequencing on formalin-fixed paraffin-embedded heart tissue specimens to detect pathogenic agents. We first investigated 4 single-sample cases to test the feasibility of this diagnostic protocol and further 3 control-sample paired cases to improve the protocol with differential metatranscriptomics next-generation sequencing (mtNGS) analysis. RESULTS: We demonstrate that differential mtNGS allows identification of various microbials as potentially pathogenic, for example, Cutibacterium acnes, Corynebacterium aurimucosum, and Pseudomonas denitrificans, which are usually commensal in healthy individuals. Differential mtNGS also allows characterization of human host response in each individual by profiling alterations of gene expression, networked pathways, and inferred immune cell compositions, information of which is beneficial for us to understand different etiologies and immunity roles in each case. Additionally, differential mtNGS allows the identification of genetic variants in patients that may contribute to their susceptibility to particular microbial infections. CONCLUSIONS: The demonstrated power of differential mtNGS in simultaneous capture of both the infectious microbial(s) and the status of human host immune response could help us better understand the pathogenesis of complex inflammatory cardiomyopathy, if conducted on a larger scale of the population. The developed differential mtNGS method could also shed light on its translation and adoption of such a laboratory test in clinic practice, allowing for a more effective diagnosis to guide therapeutic treatment of the disease.

Adenosine A3 Receptor: From Molecular Signaling to Therapeutic Strategies for Heart Diseases.

Cardiovascular diseases (CVDs), particularly heart failure, are major contributors to early mortality globally. Heart failure poses a significant public health problem, with persistently poor long-term outcomes and an overall unsatisfactory prognosis for patients. Conventionally, treatments for heart failure have focused on lowering blood pressure; however, the development of more potent therapies targeting hemodynamic parameters presents challenges, including tolerability and safety risks, which could potentially restrict their clinical effectiveness. Adenosine has emerged as a key mediator in CVDs, acting as a retaliatory metabolite produced during cellular stress via ATP metabolism, and works as a signaling molecule regulating various physiological processes. Adenosine functions by interacting with different adenosine receptor (AR) subtypes expressed in cardiac cells, including A1AR, A2AAR, A2BAR, and A3AR. In addition to A1AR, A3AR has a multifaceted role in the cardiovascular system, since its activation contributes to reducing the damage to the heart in various pathological states, particularly ischemic heart disease, heart failure, and hypertension, although its role is not as well documented compared to other AR subtypes. Research on A3AR signaling has focused on identifying the intricate molecular mechanisms involved in CVDs through various pathways, including Gi or Gq protein-dependent signaling, ATP-sensitive potassium channels, MAPKs, and G protein-independent signaling. Several A3AR-specific agonists, such as piclidenoson and namodenoson, exert cardioprotective impacts during ischemia in the diverse animal models of heart disease. Thus, modulating A3ARs serves as a potential therapeutic approach, fueling considerable interest in developing compounds that target A3ARs as potential treatments for heart diseases.

American Heart Association Cardiogenic Shock Registry: Design and Implementation.

BACKGROUND: Cardiogenic shock is a morbid complication of heart disease that claims the lives of more than 1 in 3 patients presenting with this syndrome. Supporting a unique collaboration across clinical specialties, federal regulators, payors, and industry, the American Heart Association volunteers and staff have launched a quality improvement registry to better understand the clinical manifestations of shock phenotypes, and to benchmark the management patterns, and outcomes of patients presenting with cardiogenic shock to hospitals across the United States. METHODS: Participating hospitals will enroll consecutive hospitalized patients with cardiogenic shock, regardless of etiology or severity. Data are collected through individual reviews of medical records of sequential adult patients with cardiogenic shock. The electronic case record form was collaboratively designed with a core minimum data structure and aligned with Shock Academic Research Consortium definitions. This registry will allow participating health systems to evaluate patient-level data including diagnostic approaches, therapeutics, use of advanced monitoring and circulatory support, processes of care, complications, and in-hospital survival. Participating sites can leverage these data for onsite monitoring of outcomes and benchmarking versus other institutions. The registry was concomitantly designed to provide a high-quality longitudinal research infrastructure for pragmatic randomized trials as well as translational, clinical, and implementation research. An aggregate deidentified data set will be made available to the research community on the American Heart Association's Precision Medicine Platform. On March 31, 2022, the American Heart Association Cardiogenic Shock Registry received its first clinical records. At the time of this submission, 100 centers are participating. CONCLUSIONS: The American Heart Association Cardiogenic Shock Registry will serve as a resource using consistent data structure and definitions for the medical and research community to accelerate scientific advancement through shared learning and research resulting in improved quality of care and outcomes of shock patients.

Right-Sided Aortic Arch: A Computed Tomography Angiography Investigation, A Systematic Review with Meta-Analysis.

Background/Objectives: The right-sided aortic arch (RAA) is an uncommon variation of the aortic arch (AA), characterized by the aorta crossing over the right main bronchus. In the RAA, the descending aorta can be found on either the right or left side of the spine. The current study comprises a comprehensive retrospective computed tomography angiography (CTA) investigation into the prevalence of the RAA within the Greek population. Additionally, we will conduct a systematic review and meta-analysis to elucidate both common and rare morphological variants of the RAA. This research is significant as it sheds light on the prevalence and characteristics of the RAA in a specific population, providing valuable insights for clinical practice. Methods: Two hundred CTAs were meticulously investigated for the presence of a RAA. In addition, the PubMed, Google Scholar, and Scopus online databases were thoroughly searched for studies referring to the AA morphology. The R programming language and RStudio were used for the pooled prevalence meta-analysis, while several subgroup analyses were conducted. Results: Original study: A unique case of 200 CTAs (0.5%) was identified with an uncommon morphology. The following branches emanated from the RAA under the sequence: the right subclavian artery (RSA), the right common carotid artery (RCCA), the left common carotid artery (LCCA), and the left vertebral artery (LVA) in common origin with the aberrant left subclavian artery (ALSA). The ALSA originated from a diverticulum (of Kommerell) and followed a retroesophageal course. Systematic Review and Meta-Analysis: Sixty-two studies (72,187 total cases) met the inclusion criteria. The pooled prevalence of the RAA with a mirror-image morphology was estimated at 0.07%, and the RAA with an ALSA was estimated at <0.01%. Conclusions: AA anomalies, specifically the RAA, raise clinical interest due to their coexistence with developmental heart anomalies and possible interventional complications. Congenital heart anomalies, such as the Tetralogy of Fallot and patent foramen ovale, coexisted with RAA mirror-image morphology.

The impact of the European Society of Cardiology guidelines and whole exome sequencing on genetic testing in hereditary cardiac diseases.

In the last decade, an incredible improvement has been made in elucidating the genetic bases of cardiomyopathies. Here we report the impact of either the European Society of Cardiology (ESC) guidelines or the use of whole exome sequencing (WES) in terms of a number of variants of uncertain significance (VUS) and missed diagnoses in a series of 260 patients affected by inherited cardiac disorders. Samples were analyzed using a targeted gene panel of 128 cardiac-related genes and/or WES in a subset of patients, with a three-tier approach. Analyzing (i) only a subset of genes related to the clinical presentation, strictly following the ESC guidelines, 20.77% positive test were assessed. The incremental diagnostic rate for (ii) the whole gene panel, and (iii) the WES was 4.71% and 11.67%, respectively. The diverse analytical approaches increased the number of VUSs and incidental findings. Indeed, the use of WES highlights that there is a small percentage of syndromic conditions that standard analysis would not have detected. Moreover, the use of targeted sequencing coupled with "narrow" analytical approach prevents the detection of variants in actionable genes that could allow for preventive treatment. Our data suggest that genetic testing might aid clinicians in the diagnosis of inheritable cardiac disorders.

Impact of Subspecialty Pediatric Palliative Care on Children with Heart Disease; A Systematic Review and Meta-analysis.

While many experts in pediatric cardiology have emphasized the importance of palliative care involvement, very few studies have assessed the influence of specialty pediatric palliative care (SPPC) involvement for children with heart disease. We conducted a systematic review using keywords related to palliative care, quality of life and care-satisfaction, and heart disease. We searched PubMed, EMBASE, CINAHL, CENTRAL and Web of Science in December 2023. Screening, data extraction and methodology followed Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) recommendations. Pairs of trained reviewers independently evaluated each article. All full texts excluded from the review were hand-screened for eligible references including systematic reviews in general pediatric populations. Two reviewers independently extracted: (1) study design; (2) methodology; (2) setting; (3) population; (4) intervention/exposure and control definition; (5) outcome measures; and (6) results. Of 4059 studies screened, 9 met inclusion criteria including two with overlapping patient data. Study designs were heterogenous, including only one randomized control and two historical control trials with SPPC as a prospective intervention. Overall, there was moderate to high risk of bias. Seven were single centers studies. In combined estimates, patients who received SPPC were more likely to have advance care planning documented (RR 2.7, [95%CI 1.6, 4.7], p < 0.001) and resuscitation limits (RR 4.0, [2.0, 8.1], p < 0.001), while half as likely to have active resuscitation at end-of-life ([0.3, 0.9], p = 0.032). For parental stress, receipt of SPPC improved scores by almost half a standard deviation (RR 0.48, 95%CI 0.10, 0.86) more than controls. Ultimately, we identified a paucity of high-quality data studying the influence of SPPC; however, findings correlate with literature in other pediatric populations. Findings suggest benefits of SPPC integration for patients with heart disease and their families.

Impact of preoperative respiratory distress on outcomes of slide tracheoplasty.

OBJECTIVES: Children with congenital tracheal stenosis born in the developing world face a high risk of mortality due to limited access to proper treatment. Patients who required preoperative respiratory support were suspected to have poor survival after slide tracheoplasty; however, this was not clearly demonstrated in the previous studies. This study aims to investigate the impact of preoperative respiratory conditions on outcomes of slide tracheoplasty. METHODS: From 2016 to 2022, children who underwent slide tracheoplasty were retrospectively reviewed. Patients with respiratory distress requiring emergency operations (group A) were compared with patients in stable condition who were scheduled for surgery (group B). RESULTS: Perioperative results revealed that group A (n = 43) had a longer bypass time (P < 0.001), operation time (P = 0.01), postoperative ventilation time (P < 0.001) and length of intensive care unit stay (P = 0.00125) than group B (n = 60). The early mortality rate was 7.8%, and the actuarial 5-year survival rate was 85.3%. The cumulative incidence test revealed that group A was highly significant for overall mortality [sudistribution (SHR) 4.5; 95% confidence interval (CI) 1.23-16.4; P = 0.023]. Risk factors for overall mortality were prolonged postoperative ventilation time (hazard ratio 3.86; 95% CI 1.20-12.48; P = 0.024), bronchial stenosis (hazard ratio 5.77; 95% CI 1.72-19.31; P = 0.004), and preoperative tracheal mucositis (hazard ratio 5.67; 95% CI 1.51-21.31; P = 0.01). Four patients needed reintervention during a follow-up of 28.4 months (interquartile range 15.3-47.3). CONCLUSIONS: Preoperative respiratory distress negatively affected the outcomes of patients who required slide tracheoplasty. Therefore, early detection of congenital tracheal stenosis and aggressive slide tracheoplasty are crucial and obligatory to enhance long-term survival in this lethal congenital airway disease.

Improved Cardiac Rehabilitation Referral Rate Utilizing a Multidisciplinary Quality Improvement Team.

Introduction Cardiac rehabilitation (CR) is an underutilized resource in patients with ischemic heart disease, despite being a Class IA recommendation. In this study, a multidisciplinary quality improvement (QI) team aimed to improve CR referrals by standardizing the ordering process at our hospital system. Method By using a collaborative approach involving the electronic medical record (EMR), medical provider education, and hospital protocols, our two-hospital healthcare system was able to successfully identify barriers to CR referral rates and implement interventions for these barriers. All physicians and medical providers, including ancillary staff, were educated on the EMR order sets to improve compliance by using automated order sets in the EMR. The CR referral order in the EMR included a statement regarding the application of evidence-based medicine, and a computerized provider order entry was included as a reminder to the ordering provider. The use of EMR was monitored monthly by the QI committee. Chi-square test and odds ratios were obtained for statistical analysis. Results Through provider-EMR education and patient education on discharge, CR referral rates significantly improved from 51.2 to 87.1% (p = 0.0001) in a 12-month period. The study included 1,499 patients in total. The improvement was statistically significant regardless of patient gender, race, or insurance coverage. Additionally, subgroup analysis in this study found that prior to standardization of the ordering process, African American patients were significantly less likely to be referred to CR compared to Caucasian patients. (51.2% vs. 41.0%, p=0.01). There was no statistically significant difference in the likelihood of CR referral between Caucasian and African American patients following the intervention (84.0% vs. 78.0%, p = 0.166). Conclusion This study shows that CR is an underutilized resource and that effective QI initiatives may not only increase CR referral rates but also close the gap between racial inequities in referral rates. Future research with multi-center randomized control trials is needed to further enhance its external generalizability to other institutions.

Optimization of resources in intelligent electronic health systems based on internet of things to predict heart diseases via artificial neural network.

As a paradigm shift in tandem with the expansion of ICT, smart electronic health systems hold great promise for enhancing healthcare delivery and illness prevention efforts. These systems acquire an in-depth understanding of patient health states through the real-time collection and analysis of medical data enabled by the Internet of Things (IoT) and machine learning. With the widespread use of cutting-edge artificial intelligence and machine learning techniques, predictive analytics in medicine can assist in making the shift from a reactive to a proactive healthcare strategy. With the ability to rapidly and precisely evaluate massive amounts of data, draw intelligent conclusions, and solve difficult issues, artificial neural networks could revolutionize several industries. Two cardiac illnesses were assessed in this study using a multilayer perceptron artificial neural network that incorporated a genetic algorithm and an error-back propagation mechanism. The ability of artificial neural networks to handle consecutive time series data is crucial for optimizing resources in smart electronic health systems, especially with the increasing volume of patient information and the broad use of electronic clinical records. This requires the creation of more accurate predictive models. Through the use of Internet of Things (IoT) sensors, the proposed system gathers data, which is then used to do predictive analytics on patient history-related electronic clinical data saved in the cloud. A smart healthcare system that uses Mu-LTM (multidirectional long-term memory) to accurately monitor and predict the risk of heart disease has a coverage error of 97.94 %, an accuracy of 97.89 %, a sensitivity of 97.96 %, and a specificity of 97.99 %. In comparison to other smart heart disease prediction systems, the F1-score of 97.95 % and precision of 97.71 % is very good.

Beyond the heart: Cognitive and verbal outcomes in Arab children with congenital heart diseases.

BACKGROUND: This study investigates how congenital heart diseases (CHD) characteristics and interventions affect cognitive and verbal skills in Arab children, while also uncovering previously unexplored connections between these skills and the quality of life (QoL) scores as perceived by both children and parents. METHODS: A cross-sectional study was conducted in Jordan, involving 62 children with CHD aged 6-16. Data were collected through standardized intelligence tests (namely The Raven's Progressive Matrices Test and The Wechsler Intelligence Scale for Children) and QoL assessments. RESULTS: Sex, disease severity, cyanosis, CHD defect status, conducted operations, and types of interventions did not significantly influence cognitive scores. However, a significant difference was observed in Wechsler's scores between cyanotic and non-cyanotic children (p < .01) and between severe and moderate cases (p = .01). Further, a significant positive correlation was identified between Wechsler's Scores and QoL reported by parents (r = 0.33, p < .01). This correlation was particularly pronounced in the social and school functioning dimensions of QoL. CONCLUSIONS: This study highlights the need for personalized care approaches for children with CHDs based on their individual characteristics. While cognitive abilities did not directly correlate with children's QoL reports, a significant positive correlation between verbal skills and QoL reported by parents underscores the importance of effective communication in assessing a child's overall well-being. Future research should further examine the cognitive development in this population, employing neurocognitive investigations and longitudinal studies to gain a deeper understanding of their cognitive profiles and trajectories.

Children with Congenital Heart Diseases Exhibit Altered Deep Gray Matter Structures.

BACKGROUND AND OBJECTIVES: Children with congenital heart diseases (CHDs) have an increased risk of developing neurologic deficits, even in the absence of apparent brain pathology. The aim of this work was to compare quantitative macro- and microstructural properties of subcortical gray matter structures of pediatric CHD patients with normal appearing brain magnetic resonance imaging to healthy controls. METHODS: We retrospectively reviewed children with coarctation of the aorta (COA) and hypoplastic left heart syndrome (HLHS) admitted to our hospital. We identified 24 pediatric CHD patients (17 COA, 7 HLHS) with normal-appearing brain MRI. Using an atlas-based approach, the volume and apparent diffusion coefficient (ADC) were determined for the thalamus, caudate, putamen, pallidum, hippocampus, amygdala, nucleus accumbens, cerebral white matter, cerebral cortex, and brainstem. Multivariate statistics were used to compare the extracted values to reference values from 100 typically developing children without any known cardiac or neurological diseases. RESULTS: Multivariate analysis of covariance using the regional ADC and volume values as dependent variables and age and sex as co-variates revealed a significant difference between pediatric CHD patients and healthy controls (p < 0.001). Post-hoc comparisons demonstrated significantly reduced brain volumes in most subcortical brain regions investigated and elevated ADC values in the thalamus for children with CHD. No significant differences were found comparing children with COA and HLHS. CONCLUSIONS: Despite normal appearing brain MRI, children with CHD exhibit wide-spread macro-structural and regional micro-structural differences of subcortical brain structures compared to healthy controls, which could negatively impact neurodevelopment, leading to neurological deficits in childhood and beyond.

Study of the Histopathological Changes in the Heart in Postmortem/Autopsy Cases in Tertiary Care Center.

Context: There has been a significant increase in global cardiac-related fatalities, particularly over the last five decades. In India, cardiovascular diseases have emerged as a prevalent condition. Moreover, the incidence of sudden cardiac death has shown a steady rise worldwide. Aims: The aim of this study was to research how frequently cardiac disease was found during postmortem/autopsy cases and how it affects sudden death. Materials and Methods: The research was conducted at the Department of Pathology, SRTR, GMC, Ambajogai, India. One hundred and eighty cardiac specimens were examined from October 2019 to October 2021. Formalin-fixed heart specimens had multiple sections cut out of them at 4-5 mm intervals. The tissues underwent preparation, followed by sectioning into 4 µm thickness using paraffin. The standard staining method of hematoxylin and eosin was used to stain the samples. Different cardiac histomorphological alterations were assessed. Results: The study included 180 autopsy cases. The highest numbers of cases were observed in the age group of 31-40 years (20.2%). Triple-vessel involvement was seen in the majority of 61.5% of cases, out of 180 cases, 52.7% of cases were of hypertrophy. Conclusions: The research highlights the prevalence of cardiovascular diseases, particularly atherosclerosis and hypertrophy. Notably, sudden death cases showed a distinct distribution across the study years, with males being predominantly affected. This underscores the urgent need for preventive measures and interventions to prevalent cardiovascular conditions effectively.

Surgical Repair of Atrial Septal Defect in a Patient with 3-Methylcrotonyl-CoA Carboxylase Deficiency.

3-Methylcrotonyl-CoA carboxylase (3-MCC) deficiency is a rare autosomal recessive disease of leucine catabolism. 3-MCC deficiency may lead to metabolic decompensation under stress; however, outcomes of elective surgery requiring cardiopulmonary bypass (CPB) are unknown. We report a 4-year-old girl with asymptomatic 3-MCC deficiency and atrial septal defect (ASD) who's undergone surgical ASD repair under CPB. She was otherwise normal developmentally and medically. Although patients with 3-MCC may face metabolic crises, the ASD repair under CPB was uneventful.

Art or Artifact: Evaluating the Accuracy, Appeal, and Educational Value of AI-Generated Imagery in DALL·E 3 for Illustrating Congenital Heart Diseases.

Artificial Intelligence (AI), particularly AI-Generated Imagery, has the potential to impact medical and patient education. This research explores the use of AI-generated imagery, from text-to-images, in medical education, focusing on congenital heart diseases (CHD). Utilizing ChatGPT's DALL·E 3, the research aims to assess the accuracy and educational value of AI-created images for 20 common CHDs. In this study, we utilized DALL·E 3 to generate a comprehensive set of 110 images, comprising ten images depicting the normal human heart and five images for each of the 20 common CHDs. The generated images were evaluated by a diverse group of 33 healthcare professionals. This cohort included cardiology experts, pediatricians, non-pediatric faculty members, trainees (medical students, interns, pediatric residents), and pediatric nurses. Utilizing a structured framework, these professionals assessed each image for anatomical accuracy, the usefulness of in-picture text, its appeal to medical professionals, and the image's potential applicability in medical presentations. Each item was assessed on a Likert scale of three. The assessments produced a total of 3630 images' assessments. Most AI-generated cardiac images were rated poorly as follows: 80.8% of images were rated as anatomically incorrect or fabricated, 85.2% rated to have incorrect text labels, 78.1% rated as not usable for medical education. The nurses and medical interns were found to have a more positive perception about the AI-generated cardiac images compared to the faculty members, pediatricians, and cardiology experts. Complex congenital anomalies were found to be significantly more predicted to anatomical fabrication compared to simple cardiac anomalies. There were significant challenges identified in image generation. Based on our findings, we recommend a vigilant approach towards the use of AI-generated imagery in medical education at present, underscoring the imperative for thorough validation and the importance of collaboration across disciplines. While we advise against its immediate integration until further validations are conducted, the study advocates for future AI-models to be fine-tuned with accurate medical data, enhancing their reliability and educational utility.