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Background: As more pediatric patients become candidates for heart transplantation (HT), understanding pathological predictors of outcome and the accuracy of the pretransplantation evaluation are important to optimize utilization of scarce donor organs and improve outcomes. The authors aimed to investigate explanted heart specimens to identify pathologic predictors that may affect cardiac allograft survival after HT. Methods: Explanted pediatric hearts obtained over an 11‐year period were analyzed to understand the patient demographics, indications for transplant, and the clinical–pathological factors. Results: In this study, 149 explanted hearts, 46% congenital heart defects (CHD), were studied. CHD patients were younger and mean pulmonary artery pressure and resistance were significantly lower than in cardiomyopathy patients. Twenty‐one died or underwent retransplantation (14.1%). Survival was significantly higher in the cardiomyopathy group at all follow‐up intervals. There were more deaths and the 1‐, 5‐ and 7‐year survival was lower in patients ≤10 years of age at HT. Early rejection was significantly higher in CHD patients exposed to homograft tissue, but not late rejection. Mortality/retransplantation rate was significantly higher and allograft survival lower in CHD hearts with excessive fibrosis of one or both ventricles. Anatomic diagnosis at pathologic examination differed from the clinical diagnosis in eight cases. Conclusions: Survival was better for the cardiomyopathy group and patients >10 years at HT. Prior homograft use was associated with a higher prevalence of early rejection. Ventricular fibrosis (of explant) was a strong predictor of outcome in the CHD group. We presented several pathologic findings in explanted pediatric hearts. This study describes a large single‐center experience on the various pathologies encountered in the explanted heart specimens in the pediatric age group to identify possible pathologic predictors of outcome that could potentially influence pretransplant and/or posttransplant management. [ABSTRACT FROM AUTHOR]

Aim To investigate the effects of coronary artery stenosis on cardiac pathology and myocardial metabolomics in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). Methods Coronary and ventricular sites (anterior, lateral and posterior walls of left ventricle and anterior, posterior wall of right ventricle and interventricular septum) were studied in 35 patients with ARVC undergoing heart transplantation. Coronary artery tissue sections were stained with HE and the degree of stenosis was quantitatively analyzed; slices of ventricular tissue were stained with Masson staining, and the proportion of myocadium, fiber, and adipose tissue in the heart was quantitatively analyzed after image processing. Metabolite extraction and metabolomics analysis of cardiac tissue from coronary artery supply areas were performed. Comparative analysis was conducted on the differences in myocardium, fiber and adipose tissue, and metabolomic profiles among the group of patients without coronary artery stenosis, with mild coronary artery stenosis (<50%) and with moderate-severe coronary artery stenosis (≥50%). Results Among the 35 patients with ARVC, 10 (28. 6%) had moderate-severe coronary artery stenosis, 11 (31. 4%) had mild coronary artery stenosis, and 14 (40. 0%) had no coronary artery stenosis. The age of patients with moderate-severe coronary artery stenosis receiving heart transplantation was significantly higher than those with mild coronary artery stenosis and those without coronary artery stenosis [(48. 5±10. 7) years vs. (33. 8±10. 5) years and (31. 0±13. 4) years, P =0. 015]. There was no statistically significant difference in the proportion of myocardium, fiber and adipose tissue between patients with moderate-severe, mild coronary artery stenosis and those without coronary artery stenosis in the left ventricular anterior wall, lateral wall, posterior wall, and right ventricular anterior wall, posterior wall, and interventricular septum (P>0. 05). There were 105 metabolites were detected from the metabolic profiles, which attributed to pathways of tricarboxylic acid cycle, amino acid metabolism, purine metabolism, pyrimidine metabolism, pentose phosphate metabolism, glycolysis and gluconeogenesis. Metabolomic analysis showed few differences among the three groups. There was no significant separation of the three groups on heat or in principle component analysis. Conclusion There was no difference in the proportion of myocardium, fiber and adipose tissue of the heart and metabolomic profiles among ARVC patients with moderate-severe, mild coronary artery stenosis and without coronary artery stenosis. [ABSTRACT FROM AUTHOR]

Aims Myocardial longitudinal strain (LS) by two-dimensional (2D) speckle-tracking echocardiography has a diagnostic and prognostic role in cardiac amyloidosis (CA). Typically, the apical segments of the left ventricle (LV) are less affected by LS abnormalities, a finding called relative apical sparing (RELAPS). Whether a variable burden of CA might explain the RELAPS remains unknown. We aimed to evaluate the extent, distribution, and deposition pattern of amyloid in autopsy hearts of CA patients and to correlate the histopathology findings with 2D echocardiography. Methods and results This is a retrospective study of whole heart specimens of CA patients who died and underwent autopsy and 2D echocardiography. Amyloid burden quantification was assessed by histomorphometry in each segment at different LV levels. The LS analysis results were compared with the amyloid burden and the base-to-apex distribution. Histopathology investigation of 27 hearts with CA [immunoglobulin light chains (AL) 17 cases and transthyretin (ATTR) 10 cases] demonstrated an amyloid base-to-apex gradient. In 11 CA patients with 2D echocardiography, analysis of LS and histological amyloid burden allowed to identify different patterns: RELAPS (8 cases, 73%), with (2) or without (6) amyloid gradient, normal or mildly reduced LS with diffuse low amyloid (2, 18%), and severely reduced LS with diffuse high amyloid (1, 9%). Conclusion The typical RELAPS pattern at echocardiography is not always explained by a base-to-apex gradient of amyloid burden at histopathology, suggesting that RELAPS might be an epiphenomenon of complex interactions among amyloid infiltration, myocardial structure, and adaptation. [ABSTRACT FROM AUTHOR]

Coronavirus disease 2019 (COVID-19) infection was first discovered in December 2019 and was soon declared a worldwide pandemic. With the decrease in the severity of the deadly disease, there is a false sense of relief globally. However, there has been an increasing trend in the cases of cardiovascular and other complications of COVID-19 which has raised concern about the sequelae of this infection. The number of cases of sudden cardiac death and myocardial infection, post-COVID-19 has seen a major leap, especially in healthy people of both genders. Systemic comorbidities and immunocompromised states have a direct effect on the prognosis of patients. The SARS-CoV-2 infection not just affects the respiratory system but multi-organ involvement is seen due to this deadly virus. The heart is a vital organ that has acute- acute and long-term consequences, what is known as long-COVID, which lead to increased morbidity and mortality. There are increased cases of multiple cardiovascular problems such as blood clots, cardiovascular accidents, myocarditis, myocardial infarction, and heart failure in people after recovering from SARS-CoV-2 infection. The histopathological findings in the heart due to COVID-19 infection can be interstitial edema with the presence or absence of myocarditis, lymphocytic endotheliitis, microvascular microthrombi, thrombosis, interstitial fibrosis with no endothelins, or rarely myocarditis. While the tissue diagnosis during acute illness is less likely feasible, autopsy findings can be helpful to understand better pathophysiology of the disease and thus help in the better management of the patient. This review was performed to analyze the postmortem findings of the heart in patients infected with the SARS-COV-2 virus, to understand the effect of COVID-19 and its complications on the heart. The pathological changes in the cardiovascular system need to be explained and correlated with the clinical findings and prognosis of the patients. We also want to hypothesize that these findings, especially myocarditis lead to sudden death in the young, which gets undetected on routine investigations.

Here, we report an unusual case of left atrial myxoma presented with morphology of cavernous hemangioma supplied by the right coronary artery. Surgical resection of the left atrium myxoma was performed, and the patient experienced an uneventful recovery during hospitalization.

The purpose of this report is to provide information about the different presentations of cardiac and extra-cardiac histophilosis and, to assess the antimicrobial (ATM) susceptibility of Histophilus somni isolated from these cardiac lesions to different ATM agents commonly used for treating bovine bacterial respiratory pathogens. Eight feedlot calves, which died after suffering from food rejection, apathy, hyperthermia, cough and nasal mucous discharge, and lack of response to ATM therapy, were studied. Cardiac lesions observed at necropsy included valvular/mural endocarditis, myocardial infarction, and necrotizing myocarditis, miliar non-suppurative myocarditis, myocardic necrotic sequestrum, and/or pericarditis. Histopathological, bacteriological and molecular studies confirmed the presence of a fastidious microorganism in the affected organs. H. somni showed no resistance to most ATM tested (ceftiofur, gamithromycin, enrofloxacin, florfenicol, tilmicosin). The results obtained in this study confirmed that H. somni was the main cause of the subacute cardiac lesions associated with hyperthermia, apathy and respiratory signs observed in cattle examined in this research. These presentations must be considered by veterinary practitioners in order to establish a rational therapeutic. [ABSTRACT FROM AUTHOR]

Coronavirus disease 2019 (COVID-19) infection was first discovered in December 2019 and was soon declared a worldwide pandemic. With the decrease in the severity of the deadly disease, there is a false sense of relief globally. However, there has been an increasing trend in the cases of cardiovascular and other complications of COVID-19 which has raised concern about the sequelae of this infection. The number of cases of sudden cardiac death and myocardial infection, post-COVID-19 has seen a major leap, especially in healthy people of both genders. Systemic comorbidities and immunocompromised states have a direct effect on the prognosis of patients. The SARS-CoV-2 infection not just affects the respiratory system but multi-organ involvement is seen due to this deadly virus. The heart is a vital organ that has acute-acute and long-term consequences, what is known as long-COVID, which lead to increased morbidity and mortality. There are increased cases of multiple cardiovascular problems such as blood clots, cardiovascular accidents, myocarditis, myocardial infarction, and heart failure in people after recovering from SARS-CoV-2 infection. The histopathological findings in the heart due to COVID-19 infection can be interstitial edema with the presence or absence of myocarditis, lymphocytic endotheliitis, microvascular microthrombi, thrombosis, interstitial fibrosis with no endothelins, or rarely myocarditis. While the tissue diagnosis during acute illness is less likely feasible, autopsy findings can be helpful to understand better pathophysiology of the disease and thus help in the better management of the patient. This review was performed to analyze the postmortem findings of the heart in patients infected with the SARS-COV-2 virus, to understand the effect of COVID-19 and its complications on the heart. The pathological changes in the cardiovascular system need to be explained and correlated with the clinical findings and prognosis of the patients. We also want to hypothesize that these findings, especially myocarditis lead to sudden death in the young, which gets undetected on routine investigations. [ABSTRACT FROM AUTHOR]

The development of new diagnostic methods for the prediction, treatment, and prevention of cardiovascular diseases is an urgent task. The purpose of this study was to compare specific immunological and clinical-biochemical markers in patients with cardiovascular diseases. In accordance with the established diagnosis, three groups of participants were examined, a total of 139 people, including the control group. The first group was patients with hypertension, the second group, had a diagnosis of hypertension and coronary heart disease. In these patients, biochemical parameters of blood and antibodies to β-endorphin, bradykinin, histamine, dopamine, and serotonin, traditionally used in diagnostic practice, were compared. Deviations of 38–65% in the content of antibodies to dopamine and serotonin were found for patients of the first and second groups compared with the control. A correlation of immunological parameters with biochemical and hematological parameters was found. A direct relationship was found for the level of antibodies to dopamine and AST activity (r = 0.4, p < 0.05), to histamine and ESR concentration (r = 0.6, p < 0.05). Thus, the practical significance of the combined use of tests for assessing the violation of the immunological and biochemical homeostasis of the body of patients with heart disease has been established. Monitoring of additional immunological indicators to assess the development of the disease increases the degree of prognosis of the severity of the pathology. [ABSTRACT FROM AUTHOR]

Cases of myocarditis, diagnosed clinically by laboratory tests and imaging have been described in the context of mRNA-based anti-SARS-CoV-2 vaccination. Autopsy-based description of detailed histological features of vaccine-induced myocarditis is lacking. We describe the autopsy findings and common characteristics of myocarditis in untreated persons who received anti-SARS-CoV-2 vaccination. Standardized autopsies were performed on 25 persons who had died unexpectedly and within 20 days after anti-SARS-CoV-2 vaccination. In four patients who received a mRNA vaccination, we identified acute (epi-)myocarditis without detection of another significant disease or health constellation that may have caused an unexpected death. Histology showed patchy interstitial myocardial T-lymphocytic infiltration, predominantly of the CD4 positive subset, associated with mild myocyte damage. Overall, autopsy findings indicated death due to acute arrhythmogenic cardiac failure. Thus, myocarditis can be a potentially lethal complication following mRNA-based anti-SARS-CoV-2 vaccination. Our findings may aid in adequately diagnosing unclear cases after vaccination and in establishing a timely diagnosis in vivo, thus, providing the framework for adequate monitoring and early treatment of severe clinical cases. [ABSTRACT FROM AUTHOR]

Objectives: The clinical manifestations of COVID-19 associated cardiac complications are heterogeneous, ranging from asymptomatic to severe symptoms, including arrhythmias and cardiogenic shock. For COVID-19 patients with cardiac sequela, only a small subset of patients have myocarditis; the pathogenesis of cardiac sequela caused by SARS-CoV-2 other than microthrombi associated sequela remains to be determined. Methods: Retrospective analysis of 71 heart autopsy specimens from COVID-19 and putative COVID-19 in the NIH COVID Digital Pathology Repository. Results: The most consistent observation was localized myocardial cell death not associated with either myocarditis or microthrombi. Red blood cells were typically absent from capillaries but, when observed, were predominately in linear clusters (stacks) of adjacent cells. Conclusions: Based on our retrospective analysis, we propose that localized ischemia and subsequent cell death by anoxia contributes to the cardiac pathogenesis in some COVID-19 patients. We propose two new models predicting vasoconstriction of cardiac pericyte cells induced by elevated histamine from hyper-activated mast cells or direct infection. We propose that impeded blood flow and cell death by anoxia are initial steps in the development of SARS-CoV-2 induced cardiac injury in COVID-19 patients independent of microthrombi or myocarditis.

Cardiac magnetic resonance imaging (MRI) is an essential tool for the study of hypertrophic cardiomyopathies (HCM) and for differentiating HCM from conditions with increased ventricular wall thickness, such as cardiac storage diseases. Although cardiac MRI is already used for the diagnosis and characterization of some forms of storage diseases involving the myocardium, it has not yet been used to study myocardial involvement in neuronal ceroid lipofuscinosis (NCL). Here, we describe comprehensive cardiac MRI findings in a patient with the CLN3 form of NCL showing basal inferior interventricular septal hypertrophy with maintained indexed LV mass within reference values and low T1-native values. MRI findings support a finding of abnormal storage material within themyocardiumin CLN3 disease. We recommend the possible routine use of cardiac MRI for early diagnosis of cardiac involvement in CLN3 disease (also termed juvenile NCL) and to monitor the effects of emerging CLN3 therapies on the myocardium as well. [ABSTRACT FROM AUTHOR]

Many postmortem studies address the cardiovascular effects of COVID-19 and provide valuable information, but are limited by their small sample size. The aim of this systematic review is to better understand the various aspects of the cardiovascular complications of COVID-19 by pooling data from a large number of autopsy studies. We searched the online databases Ovid EBM Reviews, Ovid Embase, Ovid Medline, Scopus, and Web of Science for concepts of autopsy or histopathology combined with COVID-19, published between database inception and February 2021. We also searched for unpublished manuscripts using the medRxiv services operated by Cold Spring Harbor Laboratory. Articles were considered eligible for inclusion if they reported human postmortem cardiovascular findings among individuals with a confirmed SARS coronavirus type 2 (CoV-2) infection. Confirmed COVID-19 patients with post-mortem cardiovascular findings. None. Studies were individually assessed for risk of selection, detection, and reporting biases. The median prevalence of different autopsy findings with associated interquartile ranges (IQRs). This review cohort contained 50 studies including 548 hearts. The median age of the deceased was 69 years. The most prevalent acute cardiovascular findings were myocardial necrosis (median: 100.0%; IQR, 20%–100%; number of studies = 9; number of patients = 64) and myocardial oedema (median: 55.5%; IQR, 19.5%–92.5%; number of studies = 4; number of patients = 46). The median reported prevalence of extensive, focal active, and multifocal myocarditis were all 0.0%. The most prevalent chronic changes were myocyte hypertrophy (median: 69.0%; IQR, 46.8%–92.1%) and fibrosis (median: 35.0%; IQR, 35.0%–90.5%). SARS-CoV-2 was detected in the myocardium with median prevalence of 60.8% (IQR 40.4-95.6%). Our systematic review confirmed the high prevalence of acute and chronic cardiac pathologies in COVID-19 and SARS-CoV-2 cardiac tropism, as well as the low prevalence of myocarditis in COVID-19. [ABSTRACT FROM AUTHOR]

Cardiac magnetic resonance imaging (MRI) is an essential tool for the study of hypertrophic cardiomyopathies (HCM) and for differentiating HCM from conditions with increased ventricular wall thickness, such as cardiac storage diseases. Although cardiac MRI is already used for the diagnosis and characterization of some forms of storage diseases involving the myocardium, it has not yet been used to study myocardial involvement in neuronal ceroid lipofuscinosis (NCL). Here, we describe comprehensive cardiac MRI findings in a patient with the CLN3 form of NCL showing basal inferior interventricular septal hypertrophy with maintained indexed LV mass within reference values and low T1-native values. MRI findings support a finding of abnormal storage material within the myocardium in CLN3 disease. We recommend the possible routine use of cardiac MRI for early diagnosis of cardiac involvement in CLN3 disease (also termed juvenile NCL) and to monitor the effects of emerging CLN3 therapies on the myocardium as well.

An anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), a rare congenital anomaly, the enlarged right coronary artery supplies the left ventricle through retrograde collaterals before preferentially directing into the lower-pressure pulmonary artery system, resulting in coronary steal. The infant type is characterized by the absence of collateral vessels, and global myocardial ischemia is a major cause of death in infancy. If not treated, up to 90% of them will die during the 1st year of life. Myocardial ischemia or infarction left ventricular (LV) dysfunction with or without mitral regurgitation, life-threatening arrhythmias, or sudden cardiac death may all be manifestations of symptomatic adult-type ALCAPA. In those patients who have survived to adulthood without surgery, pathophysiological mechanisms enabling adequate LV perfusion, such as a large number of well-formed functioning collaterals, and in a few cases, the presence of ostial stenosis of the left coronary artery (LCA), provide selective survival advantage. We review the correlation between clinical presentation, pathophysiological findings, and angiographic features of ostial stenosis of LCA in the setting of adult-type ALCAPA.

• Electron microscopy (EM) interpretation is a lost art, even in academic centers. • There remain situations in which EM is the best or only ancillary test to ascertain a specific diagnosis. • This article reviews EM as it pertains to myocardial tissue and provides illustrative examples of the spectrum of ultrastructural cardiac pathology seen in storage/metabolic diseases, cardiomyopathies, infiltrative disorders, and cardiotoxicities. Electron microscopy (EM) was a popular diagnostic tool in the 1970s and early 80s. With the adoption of newer, less expensive techniques, such as immunohistochemistry, the role of EM in diagnostic surgical pathology has dwindled substantially. Nowadays, even in academic centers, EM interpretation is relegated to renal pathologists and the handful of (aging) pathologists with experience using the technique. As such, EM interpretation is truly arcane—understood by few and mysterious to many. Nevertheless, there remain situations in which EM is the best or only ancillary test to ascertain a specific diagnosis. Thus, there remains a critical need for the younger generation of surgical pathologists to learn EM interpretation. Recognizing this need, cardiac EM was made the theme of the Cardiovascular Evening Specialty Conference at the 2023 United States and Canadian Academy of Pathology (USCAP) annual meeting in New Orleans, Louisiana. Each of the speakers contributed their part to this article, the purpose of which is to review EM as it pertains to myocardial tissue and provide illustrative examples of the spectrum of ultrastructural cardiac pathology seen in storage/metabolic diseases, cardiomyopathies, infiltrative disorders, and cardiotoxicities. [ABSTRACT FROM AUTHOR]

• Early cardiac pathology likely caused by vasoconstriction ischemia rather than anoxia • Ischemia and anoxia potentially caused by vasoconstriction of cardiac pericyte cells • Vasoconstrictions likely caused by histamine or SARS-CoV-2 pericyte infection The clinical manifestations of COVID-19 associated cardiac complications are heterogeneous, ranging from asymptomatic to severe symptoms, including arrhythmias and cardiogenic shock. For COVID-19 patients with cardiac sequela, only a small subset of patients have myocarditis; the pathogenesis of cardiac sequela caused by SARS-CoV-2 other than microthrombi associated sequela remains to be determined. Retrospective analysis of 71 heart autopsy specimens from COVID-19 and putative COVID-19 in the NIH COVID Digital Pathology Repository. The most consistent observation was localized myocardial cell death not associated with either myocarditis or microthrombi. Red blood cells were typically absent from capillaries but, when observed, were predominately in linear clusters (stacks) of adjacent cells. Based on our retrospective analysis, we propose that localized ischemia and subsequent cell death by anoxia contributes to the cardiac pathogenesis in some COVID-19 patients. We propose two new models predicting vasoconstriction of cardiac pericyte cells induced by elevated histamine from hyper-activated mast cells or direct infection. We propose that impeded blood flow and cell death by anoxia are initial steps in the development of SARS-CoV-2 induced cardiac injury in COVID-19 patients independent of microthrombi or myocarditis. [ABSTRACT FROM AUTHOR]

The role of SARS-CoV-2 as a direct cause in the cardiac lesions in patients with severe COVID-19 remains to be established. Our objective is to report the pathological findings in cardiac samples of 30 patients who died after a prolonged hospital stay due to Sars-Cov-2 infection. We performed macroscopic, histological and immunohistochemical analysis of the hearts of 30 patients; and detected Sars-Cov-2 RNA by RT-PCR in the cardiac tissue samples. The median age of our cohort was 69.5 years and 76.6% were male. The median time between symptoms onset and death was 36.5 days. The main comorbidities were arterial hypertension (13 patients, 43.3%), dyslipidemia (11 patients, 36.7%), cardiovascular conditions (8 patients, 26.7%), and obesity (8 patients, 26.7%). Cardiovascular conditions included ischemic cardiopathy in 4 patients (13.3%), hypertrophic cardiomyopathy in 2 patients (6.7%) and valve replacement and chronic heart failure in one patient each (3.3%). At autopsy, the most frequent histopathological findings were coronary artery atherosclerosis (8 patients, 26.7%), left ventricular hypertrophy (4 patients, 13.3%), chronic epicardial inflammation (3 patients, 10%) and adipose metaplasia (2 patients, 6.7%). Two patients showed focal myocarditis, one due to invasive aspergillosis. One additional patient showed senile amyloidosis. Sars-Cov-2 RNA was detected in the heart of only one out of 30 patients, who had the shortest disease evolution of the series (9 days). However, no relevant cardiac histological alterations were identified. In present series, cardiac pathology was only modest in most patients with severe COVID-19. At present, the contribution of a direct effect of SARS-CoV-2 on cardiac lesions remains to be established.

Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

An anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), a rare congenital anomaly, the enlarged right coronary artery supplies the left ventricle through retrograde collaterals before preferentially directing into the lower-pressure pulmonary artery system, resulting in coronary steal. The infant type is characterized by the absence of collateral vessels, and global myocardial ischemia is a major cause of death in infancy. If not treated, up to 90% of them will die during the 1st year of life. Myocardial ischemia or infarction left ventricular (LV) dysfunction with or without mitral regurgitation, life-threatening arrhythmias, or sudden cardiac death may all be manifestations of symptomatic adult-type ALCAPA. In those patients who have survived to adulthood without surgery, pathophysiological mechanisms enabling adequate LV perfusion, such as a large number of well-formed functioning collaterals, and in a few cases, the presence of ostial stenosis of the left coronary artery (LCA), provide selective survival advantage. We review the correlation between clinical presentation, pathophysiological findings, and angiographic features of ostial stenosis of LCA in the setting of adult-type ALCAPA. [ABSTRACT FROM AUTHOR]

Primary malignant pericardial mesothelioma is a rare cardiac neoplasm. The authors evaluated risk factors, clinical presentation, and outcomes by reviewing all biopsy-confirmed cases at one institution. The use of multimodality imaging, detailed hemodynamic assessment for the presence of an effusive-constrictive profile, and cytology evaluation can support the diagnosis. (Level of Difficulty: Advanced.)

Anabolic androgenic steroids (AAS) have several adverse effects on the cardiovascular system that may lead to a sudden cardiac death (SCD). We herein report a case involving a 24-year-old male, AAS abuser with intramuscular delivery in the 6 months before, who suffered a cardiorespiratory arrest at home’s bathtub when returning from New Year’s party. A forensic autopsy was performed according to the guidelines of the Association for European Cardiovascular Pathology (AECVP). The body showed hypertrophy of skeletal musculature, with low amount of subcutaneous fat and no signs of injury (body mass index, BMI: 26.8 kg/m2). On internal examination, there were multiorgan congestion, acute pulmonary edema, and cardiomegaly (420 g) with severe coronary atherosclerosis and superimposed acute occlusive thrombosis at the left main trunk and left anterior descendant. Areas of scarring were located at the intersection between the posterior wall and the posterior third of the septum (postero-septal). At histology, acute myocardial infarction at the anterior third of the septum and the anterior wall, and subacute myocardial infarction at apical septum and apical posterior wall were detected. Other findings were small intramyocardial vessel disease and myocytes hypertrophy. Chemicotoxicological analysis in blood showed ethanol ((0.90 ± 0.05) g/L), stanazolol (11.31 µg/L), nandrolone (2.05 µg/L) and testosterone (

Abstract Background Partial anomalous pulmonary venous return is a rare congenital cardiac anomaly that usually involves the right pulmonary vein and an atrial septal defect. Isolated partial anomalous pulmonary venous return with an intact atrial septum is even rarer, and this condition is usually treated surgically in younger patients. We describe isolated partial anomalous pulmonary venous return in a 65-year-old woman who was treated by caval division with pericardial patch baffling through a surgically created atrial septal defect and reconstruction of the superior vena cava using a prosthetic graft. Case presentation A 65-year-old Asian woman who presented with exertional dyspnea was diagnosed with isolated partial anomalous pulmonary venous return. The surgical indications and strategy were controversial because of the rarity of this pathology. She had an indication for surgery because she was symptomatic and had a high ratio of pulmonary to systemic blood flow. We considered that surgical procedures should avoid postoperative stenosis of a reconstructed flow tract, sinus node dysfunction, and thrombogenesis. We created a caval division with pericardial patch baffling through a surgically created atrial septal defect and reconstructed the superior vena cava using a prosthetic graft for the isolated partial anomalous pulmonary venous return. She has since remained free of exertional dyspnea, arrhythmia, and thrombotic complications. This surgical strategy is safe and effective for treating isolated partial anomalous pulmonary venous return in older symptomatic adults. Conclusions The long-term outcome of surgical repair of partial anomalous pulmonary venous return with an intact atrial septum in our patient, a symptomatic 65-year-old woman, was excellent.

Pregnancy-related hypertension is a major cause of maternal and fetal mortality worldwide. About 10% of maternal mortality in Asia and Africa is due to high blood pressure in pregnancy. In Latin America, hypertensive disorders in pregnancy are responsible for 25% of maternal mortality.(1) The paper aims to evaluate the incidence of hypertensive disorders associated with the pregnancy between January 2019 and December 2019 at the Obstetrics and Gynaecology Clinic of the County Emergency Clinical Hospital in Sibiu, Romania. Between January 2019 and December 2019, in the Obstetrics and Gynecology clinic of the Sibiu County Emergency Clinical Hospital, 69 pregnant women with pregnancy-related hypertensive pathology were hospitalized. Of these, 95,65% of pregnant women had a single fetal pregnancy and 4,34% had a twin pregnancy. The patients were between 16 and 44 years old. In the first age category, there was only one pregnant woman who developed hypertensive pathology in pregnancy. The second age category included 50 pregnant women, while the third age category included 18 pregnant women. The average age parameter at the time of admission was 30.79 years old. [ABSTRACT FROM AUTHOR]

We perform unsupervised analysis of image-derived shape and motion features extracted from 3,822 cardiac Magnetic resonance imaging (MRIs) of the UK Biobank. First, with a feature extraction method previously published based on deep learning models, we extract from each case 9 feature values characterizing both the cardiac shape and motion. Second, a feature selection is performed to remove highly correlated feature pairs. Third, clustering is carried out using a Gaussian mixture model on the selected features. After analysis, we identify 2 small clusters that probably correspond to 2 pathological categories. Further confirmation using a trained classification model and dimensionality reduction tools is carried out to support this finding. Moreover, we examine the differences between the other large clusters and compare our measures with the ground truth.

Background: A high shock index (SI), the ratio of heart rate (HR) to systolic blood pressure (SBP), has been associated with unfavorable outcomes. We sought to determine the hemodynamic underpinnings of an elevated SI using 2-D and doppler Transthoracic Echocardiography (TTE) in unselected cardiac intensive care unit (CICU) patients., Methods: We included Mayo Clinic CICU admissions from 2007 to 2018 who were in sinus rhythm at the time of TTE. The SI was calculated using HR and SBP at the time of TTE. Patients were grouped according to SI: <0.7, 4012 (64%); 0.7-0.99, 1764 (28%); and ≥ 1.0, 513 (8%). Pearson's correlation coefficient was used to assess associations between continuous variables., Results: We included 6289 unique CICU patients, 58% of whom had acute coronary syndrome. The median age was 67.9 years old and 37.8% were females. The mean SI was 0.67 BPM/mmHg. As the SI increased, markers of left ventricular (LV) systolic function and forward flow decreased, including left ventricular ejection fraction (LVEF), fractional shortening, left ventricular outflow tract (LVOT) velocity time integral (VTI), stroke volume, LV stroke work index, and cardiac power output. Biventricular filling pressures increased, and markers of right ventricular function worsened with rising SI. Most TTE measurements reflecting LV function and forward flow were inversely correlated with SI, including LV stroke work index (r = -0.59) and LVOT VTI (r = -0.41), as were both systemic vascular resistance index (r = -0.43) and LVEF (r = -0.23)., Conclusion: CICU patients with elevated SI have worse biventricular function and systemic hemodynamics, particularly decreased stroke volume and related calculated TTE parameters. The SI is an easily available marker that can be used to identify CICU patients with unfavorable hemodynamics who may require further assessment., Competing Interests: Declaration of Competing Interest The authors have no relevant conflicts of interest., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: Systemic diseases, diabetes mellitus (DM), and cardiovascular disease (CaVD) have been suggested being risk factors for cognitive impairment (CI) and/or influence Alzheimer's disease neuropathologic change (ADNC).Objective: The purpose was to assess the type and the extent of neuropathological alterations in the brain and to assess whether brain pathology was associated with CaVD or DM related alterations in peripheral organs, i.e., vessels, heart, and kidney.Methods: 119 subjects, 15% with DM and 24% with CI, age range 80 to 89 years, were chosen and neuropathological alterations were assessed applying immunohistochemistry.Results: Hyperphosphorylated τ (HPτ) was seen in 99%, amyloid-β (Aβ) in 71%, transactive DNA binding protein 43 (TDP43) in 62%, and α-synuclein (αS) in 21% of the subjects. Primary age related tauopathy was diagnosed in 29% (more common in females), limbic predominant age-related TDP encephalopathy in 4% (14% of subjects with CI), and dementia with Lewy bodies in 3% (14% of subjects with CI) of the subjects. High/intermediate level of ADNC was seen in 47% and the extent of HPτ increased with age. The extent of ADNC was not associated with the extent of pathology observed in peripheral organs, i.e., DM or CaVD. Contrary, brain alterations such as pTDP43 and cerebrovascular lesions (CeVL) were influenced by DM, and CeVL correlated significantly with the extent of vessel pathology.Conclusion: In most (66%) subjects with CI, the cause of impairment was "mixed pathology", i.e., ADNC combined with TDP43, αS, or vascular brain lesions. Furthermore, our results suggest that systemic diseases, DM and CaVD, are risk factors for CI but not related to ADNC. [ABSTRACT FROM AUTHOR]

Myotonic dystrophy type 1 (DM1), the most common muscular dystrophy affecting adults and children, is a multi-systemic disorder affecting skeletal, cardiac, and smooth muscles as well as neurologic, endocrine and other systems. This review is on the cardiac pathology associated with DM1. The heart is one of the primary organs affected in DM1. Cardiac conduction defects are seen in up to 75% of adult DM1 cases and sudden death due to cardiac arrhythmias is one of the most common causes of death in DM1. Unfortunately, the pathogenesis of cardiac manifestations in DM1 is ill defined. In this review, we provide an overview of the history of cardiac studies in DM1, clinical manifestations, and pathology of the heart in DM1. This is followed by a discussion of emerging data about the utility of cardiac magnetic resonance imaging (CMR) as a biomarker for cardiac disease in DM1, and ends with a discussion on models of cardiac RNA toxicity in DM1 and recent clinical guidelines for cardiologic management of individuals with DM1.

CHRONIC KIDNEY DISEASE (CKD) IS A PATHOLOGY THAT CAN LEAD TO SLOW LOSS OF NEPHRONS AND RENAL FUNCTION, WITH THE ONSET OF TERMINAL CKD. THE PATHOPHYSIOLOGICAL CHANGES ARE COMPLEX, AND THE ASSOCIATION, ADVERSE, WITH A LARGE CARDIO-VASCULAR PATHOLOGY, CAN LEAD TO THE DEVELOPMENT OF SEPSIS LATER WITH THE RAPID DECLINE OF RENAL FUNCTION. THE CASE OF A MALE PATIENT WITH KNOWN CARDIAC PATHOLOGY, RECENTLY DISCHARGED FROM THE CARDIOLOGY CLINIC, WHO PRESENTS AT THE ER (EMERGENCY ROOM), WITH A VERY SERIOUS GENERAL CONDITION, WITH RESPIRATORY PATHOLOGY, DYSPNEA WITH ORTHOPNEA, OEDEMA OF THE LOWER LIMBS, PALE TEGUMENTS, SWEAT, BILATERAL SUCCESSIVE RALES, REASONS FOR BEING ADMITTED TO THE CARDIOLOGY SECTION, FALL INTO THIS CATEGORY. AFTER MULTIPLE CLINIC-PARACLINICAL INVESTIGATIONS WERE PERFORMED IN THE CLINIC, THE GENERAL CONDITION OF THE PATIENT WORSENED AFTER APPROXIMATELY 10 DAYS, WHICH IS WHY HE IS TRANSFERRED TO THE INTENSIVE CARE CLINIC, WHERE FURTHER INVESTIGATIONS ARE CARRIED OUT, TREATING THE FACTORS THAT HAVE WORSENED THE PATIENT'S CONDITION, INCLUDING TREATMENT OF ACUTE CKD AND CARRYING OUT THERAPY FOR RENAL FUNCTION REPLACEMENT BY HEMODIALYSIS (HD). HEMODIALYSIS WAS A USEFUL AND LIFE-SAVING THERAPEUTIC MEASURE, CHRONIC KIDNEY DISEASE, ASSOCIATED WITH A SERIOUS CARDIAC PATHOLOGY, FAVOURING THE ONSET OF SEPSIS. [ABSTRACT FROM AUTHOR]

We herein report the long-term changes in cardiac function and pathological findings after successful explantation of a left ventricular assist device in a 42-year-old patient with anthracycline-induced cardiomyopathy with reworsening heart failure. Endomyocardial biopsy samples revealed that the cardiomyocyte diameter decreased and collagen volume fraction increased just after left ventricular assist device explantation. The collagen volume fraction decreased after 6 months, despite preserved systolic function. At 5 years after left ventricular assist device explantation, the systolic function markedly decreased and cardiomyocyte diameter increased. Pathological changes of the myocardium may enable the identification of cardiac dysfunction prior to echocardiographic changes in patients with reworsening heart failure after left ventricular assist device explantation. [ABSTRACT FROM AUTHOR]

Background: Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary anomaly that results in high mortality if left untreated. Our aim was to extend our knowledge of the histological, angiographic, and clinical characteristics of ALCAPA in order to deepen our understanding of this rare entity.Case Presentation: We were involved in the assessment, treatment, and pathological evaluation of two adult ALCAPA patients who were rescued from ventricular fibrillation and then surgically treated to establish a dual coronary artery system. Histological studies indicated various chronic ischemic changes in the myocardium, patchy fibrosis, and severely thickened arteriolar walls in both ventricles. The first patient is alive and well 11.5 years after surgical correction without any implantable cardioverter defibrillator (ICD) activations. The second patient required re-do surgery 9 months after the initial operation but subsequently died. Histologically, chronic ischemic alteration of the myocardium and thickened arteriolar walls persisted even after surgical correction, and coronary angiography (CAG) showed an extremely slow flow phenomenon even after surgical correction in both patients. The average postoperative opacification rate in the first case was 7.36 + 1.12 (n = 2) in the RCA, 3.81 + 0.51 (n = 3) in the left anterior descending (LAD) artery, and 4.08 + 0.27 (n = 4) in the left circumflex (LCx) artery. The slow flow phenomenon may represent persistent high arteriolar resistance in both ventricles.Conclusions: Seldom reported or new findings in adult ALCAPA were identified in two cases. More frequent diagnosis of adult ALCAPA can be expected because of the widespread availability of resuscitation and more advanced diagnostic modalities. Accumulation of pathological and clinical findings and confirmation of the long-term follow-up results after treatment may contribute to expanding our knowledge of this rare entity and establishing optimal treatment. [ABSTRACT FROM AUTHOR]

Sudden cardiac death is a significant cause of mortality in adults with congenital heart disease (CHD). The Cook County Medical Examiner's Office database was queried for cases of CHD as a cause of death in the period between July 2008 and April 2019. Twenty‐two cases were identified, including 11 decedents with simple defects and 10 decedents with complex defects. All of the subjects were in apparent good health at the time of death. In the absence of other obvious causes of death, simple defects were considered cases of sudden cardiac death. Significant cardiac morphological changes were common in complex defects. While 16 cases had known, diagnosed/treated CHD, 5 cases had no diagnosis prior to autopsy. In these cases, the ability to recognize CHD (sometimes subtle) helped in determining the causes of death. Therefore, forensic pathologists must be able to properly recognize various forms of CHD and request consultations, when needed. [ABSTRACT FROM AUTHOR]

Abstract Background Hereditary proximal spinal muscular atrophy (SMA) is a severe neuromuscular disease of childhood caused by homozygous loss of function of the survival motor neuron (SMN) 1 gene. The presence of a second, nearly identical SMN gene (SMN2) in the human genome ensures production of residual levels of the ubiquitously expressed SMN protein. Alpha-motor neurons in the ventral horns of the spinal cord are most vulnerable to reduced SMN concentrations but the development or function of other tissues may also be affected, and cardiovascular abnormalities have frequently been reported both in patients and SMA mouse models. Methods We systematically reviewed reported cardiac pathology in relation to SMN deficiency. To investigate the relevance of the possible association in more detail, we used clinical classification systems to characterize structural cardiac defects and arrhythmias. Conclusions Seventy-two studies with a total of 264 SMA patients with reported cardiac pathology were identified, along with 14 publications on SMA mouse models with abnormalities of the heart. Structural cardiac pathology, mainly septal defects and abnormalities of the cardiac outflow tract, was reported predominantly in the most severely affected patients (i.e. SMA type 1). Cardiac rhythm disorders were most frequently reported in patients with milder SMA types (e.g. SMA type 3). All included studies lacked control groups and a standardized approach for cardiac evaluation. The convergence to specific abnormalities of cardiac structure and function may indicate vulnerability of specific cell types or developmental processes relevant for cardiogenesis. Future studies would benefit from a controlled and standardized approach for cardiac evaluation in patients with SMA.

The recent special issue of the World Journal for Pediatric and Congenital Heart Surgery devoted to hypoplastic left heart syndrome, and its related anomalies, contained significant information of great clinical relevance. Very little attention, however, was devoted to the integrity of ventricular septum as providing a criterion to distinguish between the phenotypes to be included within the syndrome, as opposed to the related anomalies. In this commentary, we summarize the evidence in support of the notion that the phenotypes to be included within the syndrome can be interpreted on the basis of an acquired disease of fetal life. We suggest that it is the integrity of the ventricular septum that provided the major criterion for the distinction between the lesions making up the syndrome and the related anomalies. The subsets of lesions to be included within the syndrome can then be recognized in terms of the time, subsequent to the closure of the embryonic interventricular communication, at which the left ventricle ceased its growth relative to the remainder of the cardiac components. On this basis, it is possible to recognize the combinations of aortic and mitral atresia, mitral stenosis with aortic atresia, combined mitral and aortic stenosis, and hypoplasia of the left ventricle with commensurate hypoplasia of the aortic and mitral valves; the latter combination now recognized as the hypoplastic left heart complex., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

In patients with left ventricular assist device (LVAD), a minority of post-operative ventricular tachycardias (VTs) is caused by contact between the inflow cannula and the endocardium. Currently, electrophysiologic characteristics and pathologic features of this condition are lacking. We report on a case of a successfully ablated mechanical VT. After VT recurrence, heart transplantation took place. Pathologic observations were consistent with direct tissue injury and inflammation, eventually contributing to persisting arrhythmias. Radiofrequency catheter ablation can be a safe and effective option to treat arrhythmias caused by inflow cannula interference in the short term, although a high recurrence rate is expected.

Anabolic androgenic steroids (AAS) have several adverse effects on the cardiovascular system that may lead to a sudden cardiac death (SCD). We herein report a case involving a 24-year-old male, AAS abuser with intramuscular delivery in the 6 months before, who suffered a cardiorespiratory arrest at home's bathtub when returning from New Year's party. A forensic autopsy was performed according to the guidelines of the Association for European Cardiovascular Pathology (AECVP). The body showed hypertrophy of skeletal musculature, with low amount of subcutaneous fat and no signs of injury (body mass index, BMI: 26.8 kg/m2). On internal examination, there were multiorgan congestion, acute pulmonary edema, and cardiomegaly (420 g) with severe coronary atherosclerosis and superimposed acute occlusive thrombosis at the left main trunk and left anterior descendant. Areas of scarring were located at the intersection between the posterior wall and the posterior third of the septum (postero-septal). At histology, acute myocardial infarction at the anterior third of the septum and the anterior wall, and subacute myocardial infarction at apical septum and apical posterior wall were detected. Other findings were small intramyocardial vessel disease and myocytes hypertrophy. Chemicotoxicological analysis in blood showed ethanol ((0.90 ± 0.05) g/L), stanazolol (11.31 µg/L), nandrolone (2.05 µg/L) and testosterone (<1.00 µg/L). When confronted with a sudden death in a young athlete we must pay attention to the physical phenotype that may suggest AAS abuse and perform a detailed examination of the heart. Chemicotoxicological analysis is a key to establish the relationship between SCD and AAS abuse. [ABSTRACT FROM AUTHOR]

• A novel semi-supervised learning method is developed for apparent flow estimation. • Combining this flow with segmentation, motion-characteristic features are extracted. • An explainable and simple cardiac pathology classification model is proposed. We propose a method to classify cardiac pathology based on a novel approach to extract image derived features to characterize the shape and motion of the heart. An original semi-supervised learning procedure, which makes efficient use of a large amount of non-segmented images and a small amount of images segmented manually by experts, is developed to generate pixel-wise apparent flow between two time points of a 2D+t cine MRI image sequence. Combining the apparent flow maps and cardiac segmentation masks, we obtain a local apparent flow corresponding to the 2D motion of myocardium and ventricular cavities. This leads to the generation of time series of the radius and thickness of myocardial segments to represent cardiac motion. These time series of motion features are reliable and explainable characteristics of pathological cardiac motion. Furthermore, they are combined with shape-related features to classify cardiac pathologies. Using only nine feature values as input, we propose an explainable, simple and flexible model for pathology classification. On ACDC training set and testing set, the model achieves 95% and 94% respectively as classification accuracy. Its performance is hence comparable to that of the state-of-the-art. Comparison with various other models is performed to outline some advantages of our model. [ABSTRACT FROM AUTHOR]