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In this review, recent key publications related to acute coronary syndrome (ACS) are summarized and placed into context of contemporary practice. Landmark trials examining vascular access in ST-elevation myocardial infarction, the management of multivessel disease, acute myocardial infarction and cardiac arrest are discussed. An update in pharmacology for ACS provides updates in major trials relating to P2Y12 inhibitor initiation, deescalation, and use in special populations. Additional updates in the use of lipid-lowering agents and adjunctive medications in ACS are reviewed. Finally, cardiac pathology related to coronavirus disease 2019 (COVID-19), as well as the impact of the COVID-19 global pandemic on the care of patients with ACS, is summarized.

OBJECTIVE Based on cybernetics, a large system can be divided into subsystems, and the stability of each can determine the overall properties of the system. However, this stability analysis perspective has not yet been employed in electrocardiogram (ECG) signals. This is the first study to attempt to evaluate whether the stability of decomposed ECG subsystems can be analyzed in order to effectively investigate the overall performance of ECG signals, and aid in disease diagnosis. METHODS We used seven different cardiac pathologies (myocardial infarction, cardiomyopathy, bundle branch block, dysrhythmia, hypertrophy, myocarditis, and valvular heart disease) to illustrate our method. Dynamic mode decomposition (DMD) was first used to decompose ECG signals into dynamic modes (DMs) which can be regarded as ECG subsystems. Then, the features related to the DMs stabilities were extracted, and nine common classifiers were implemented for classification of these pathologies. RESULTS Most features were significant for differentiating the above-mentioned groups (value

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.

We developed a video-enhanced preparticipation evaluation symptom questionnaire (the V-PPE), intended to help screen athletes for heart disease. We now report results of a pilot quality improvement study evaluating V-PPE's performance. In a prospective before-and-after study, approximately 5700 high-school athletes were prompted to voluntarily fill out the V-PPE questionnaire. We compared symptom frequencies on standard PPE to those on V-PPE. Of 5700 athletes, 46 (0.8%), 117 (2.0%), 33 (0.6%), and 101 (1.8%) reported syncope, angina, palpitations, and dyspnea, respectively on routine screening. Four hundred and ninety-two (8.6%) voluntarily filled out the V-PPE. Athletes were more likely to report palpitations on V-PPE than PPE, but not angina, dyspnea, syncope, or at least one symptom. Symptom frequencies on electronic PPE questionnaires are lower than recent reports suggest. Embedded videos can alter screening yield. More research is necessary to evaluate the predictive value of the V-PPE for clinically relevant cardiac pathology.

Context Emergency medicine (EM) physicians commonly stabilize patients with acute psychiatric distress, such as suicidal ideation. Research has shown that suicidal ideation is difficult to manage in emergency department (ED) settings and that patients in psychiatric distress are often “boarded” in the ED while awaiting more definitive care. Objectives To examine the attitudes and experiences of emergency physicians regarding the care of patients in psychiatric distress. Special attention is given to suicidal ideation due to its prevalence in the United States. Methods A 19 question anonymous survey was sent via email to 55 emergency medicine residency directors throughout Michigan, Ohio, Indiana, and Illinois, who were identified using an Internet search of residency programs in the region. The program directors were asked to distribute the survey to their colleagues and residents. The intent of this procedure was to generate as many survey responses as possible, while obscuring the identities of the respondents. Responses were gathered from October 29, 2019 until January 16, 2020. The survey was designed to assess respondents’ self-reported demographic data as well as their experiences with the boarding process, initial examination, final disposition, reevaluation of the patient, physician training and resources, and follow up care. Statistical analysis was performed using a Mann–Whitney U test, significance was set at p Results In total, 47 EM physicians responded to the survey; however, not all of the respondents completed all 19 questions. Ten of 44 respondents (22.7%) reported that they do not perform the initial psychiatric examination themselves and instead defer to a nurse or social worker. Twenty-two of 44 respondents (50.0%) reported that they defer to a social worker when determining the final disposition of psychiatric patients. Respondents reevaluated patients in psychiatric distress statistically significantly less often (p=0.01) compared with patients with cardiac pathology. Additionally, 15 of 38 respondents (39.5%) reported that they did not feel adequately trained to handle psychiatric emergencies, and 36 of 39 respondents (92.3%) of physicians felt that their facility would benefit from additional mental health resources. Thirty five of 39 respondents (89.7%) reported that their facility did not have a system in place to follow up with suicidal patients upon discharge. Conclusions Caring for patients who are acutely suicidal or in psychiatric distress is complex and more research is needed to optimize treatment strategies. The results of this study indicate that EM physicians may regularly defer to nonphysician providers when evaluating and treating patients in psychiatric distress. A perceived lack of training in psychiatry may contribute to this practice. The results of this study are in accord with previous research that indicated a need for additional psychiatry training in EM residencies.

The number of people involved in regular exercise and sports is increasing. Not infrequently, this is associated with intake of sports biologically active food supplements (BAS) and stimulators of physical ability. Data has been reported on the frequency of use of physical ability stimulators among professional athletes and on the use of the most popular food supplements among young people. Special attention is paid to the effect of such use on the cardiovascular system of athletes. This review describes negative cardiac effects and clinical cases of death of athletes due to the use of such supplements and stimulators.

COVID-19 has emerged as one of the most devastating and clinically significant infectious diseases of the last decade. It has reached global pandemic status at an unprecedented pace and has placed significant demands on health care systems worldwide. Although COVID-19 primarily affects the lungs, epidemiologic reports have shown that the disease affects other vital organs of the body, including the heart, vasculature, kidneys, brain, and the hematopoietic system. Of importance is the emerging awareness of the effects of COVID-19 on the cardiovascular system. The current state of knowledge regarding cardiac involvement in COVID-19 is presented in this article, with particular focus on the cardiovascular manifestations and complications of COVID-19 infection. The mechanistic insights of disease causation and the relevant pathophysiology involved in COVID-19 as they affect the heart are explored and described. Relevant practice essentials and clinical management implications for patients with COVID-19 with a cardiac pathology are presented in light of recent evidence.

Objective To decrease referrals to cardiology of patients ages 7 to 21 years with low-probability cardiac pathology who presented to primary care with chest pain by 50% within 24 months. Study Design A multidisciplinary team designed and implemented an initiative consisting of 1) a decision support tool (DST), 2) educational sessions, 3) routine feedback to improve use of referral criteria, and 4) patient family education. Four pediatric practices, comprising 34 pediatricians and 7 nurse practitioners, were included in this study. We tracked progress via statistical process control charts. Results A total of 421 patients ages 7 to 21 years presented with chest pain to their pediatrician. The utilization of the DST increased from baseline of 16% to 68%. Concurrently, the percentage of low-probability cardiology referrals in pediatric patients ages 7 to 21 years who presented with chest pain decreased from 17% to 5% after our interventions. At a median follow-up time of 0.9 years (interquartile range, 0.3–1.6 years), no patient had a life-threatening cardiac event. Conclusion Our health care improvement initiative to reduce low-probability cardiology referrals for children presenting to primary care practices with chest pain was feasible, effective, and safe.

Carvajal and erythrokeratodermia cardiomyopathy syndromes (EKC) are rare, inherited cardiocutaneous disorders with potentially fatal consequences in young children. Some patients display features of congestive heart failure and rapidly deteriorate; others exhibit no evident warning signs until sudden death reveals underlying heart disease. We present two patients to illustrate the characteristic hair, skin, teeth, and nail abnormalities, which-especially when distinct from that of other family members-should prompt cardiac evaluation and genetic analysis. In this article, we discuss established treatments as well as a promising, novel therapeutic that has led to nearly complete resolution of the cutaneous and cardiac pathology in EKC syndrome.

Cardiovascular disease is a pressing health problem with significant global health, societal, and financial burdens. Understanding the molecular basis of polygenic cardiac pathology is thus essential to devising novel approaches for management and treatment. Recent identification of uncharacterized regulatory functions for a class of nuclear envelope proteins called nucleoporins offers the opportunity to understand novel putative mechanisms of cardiac disease development and progression. Consistent reports of nucleoporin deregulation associated with ischemic and dilated cardiomyopathies, arrhythmias and valvular disorders suggests that nucleoporin impairment may be a significant but understudied variable in cardiopathologic disorders. This review discusses and converges existing literature regarding nuclear pore complex proteins and their association with cardiac pathologies, and proposes a role for nucleoporins as facilitators of cardiac disease.

The article presents modern data on the causes of platypnea, methods of its diagnosis and treatment. The data on the development of platypnea syndrome are given not only in cardiac pathology, but also in severe liver diseases with the development of hepatopulmonary syndrome and chronic obstructive pulmonary disease of a severe course.В статье изложены современные данные о причинах возникновения платипноэ, методах его диагностики и лечения. Приводятся данные о развитии синдрома платипноэ не только при патологии сердца, но и при тяжелых заболеваниях печени с развитием гепатопульмонального синдрома, хронической обструктивной болезни легких тяжелого течения.

Introduction Triathlon is one of the fastest growing sports in the United Kingdom. However, in recent years several deaths have occurred. The intention of this study is to identify these cases and examine the role cardiovascular disease played in these deaths. Methods An extensive online search was performed to identify triathlon-related deaths (TRDs) in the United Kingdom and UK citizens who died during or as a result of competing in triathlons abroad. British Triathlon provided the number of participants who took part in UK-based events. Coroners provided information on all those who died. Results Between 2009 and 2015, 991,186 participants took part in British Triathlon-sanctioned events. Five TRDs in the United Kingdom were identified. The mortality rate was 0.5 per 100,000 participants. Deaths occurred during or after the swim (3), cycle (1), and run (1) events. During the same period, 5 TRDs were identified among UK citizens competing abroad. These deaths occurred during or after the swim (2), cycle (1), and run (2) events. Cardiovascular pathology was cited as a cause or contributing factor in half of the fatalities. Four deaths were referred to a specialist cardiac pathology service for autopsy. Conclusions Cardiovascular disease was found to be the most common cause of TRD. Further research is needed to determine the underlying cardiac pathology that triggers TRDs. With this information it may be possible to develop screening tools that can prevent similar fatalities from occurring in the future.

Aims Non-acute chest pain is a common complaint and can be caused by various conditions. With the rising healthcare expenditures of today, it is necessary to use our healthcare resources effectively. This study aims to give insight into the diagnostic effort and costs for patients with non-acute chest pain. Methods and results Financial data of patients without a cardiac history from four hospitals (January 2012–October 2018), who were registered with the national diagnostic code ‘no cardiac pathology’ (ICD-10 Z13.6), ‘chest wall syndrome’ (ICD-10 R07.4), or ‘stable angina pectoris’ (ICD-10 I20.9) were extracted. In total, 74 091 patients were included for analysis and divided into the following final diagnosis groups: no cardiac pathology: N = 19 688 (age 53 ± 18), 46% male; chest wall syndrome: N = 40 858 (age 56 ± 15), 45% male; and stable angina pectoris (AP): N = 13 545 (age 67 ± 11), 61% male. A total of approximately €142.7 million was spent during diagnostic work-up. The total expenditure during diagnostic effort was €1.97, €8.13, and €10.7 million, respectively for no cardiac pathology, chest wall syndrome, and stable AP per year. After 8 years of follow-up, ≥95% of the patients diagnosed with no cardiac pathology or chest wall syndrome had an (cardiac) ischaemic-free survival. Conclusion The diagnostic expenditure and clinical effort to ascertain non-cardiac chest pain are high. We should define what we as society find acceptable as ‘assurance costs’ with an increasing pressure on the healthcare system and costs.

OBJECTIVES We evaluated 23 years of data for cornea donors at the Istanbul Faculty of Medicine Lions Eye Bank. MATERIALS AND METHODS Annual statistics of corneal donors between 1996 and 2019 were reviewed retrospectively. Records for 2008 and previous years were compared with records for 2009 and years thereafter,to assess donor demographics and reasons for discard of corneas. RESULTS A total of 3849 corneas were obtained from 2018 donors during a 23-year period. Of these, 26 donors (11.2%) were registered, whereas 1792 (88.8%) did not register any decision for donation. There were 210 (5.46%) corneas discarded for positive serology and 291 (7.56%) for unsuitable tissue morphology, and 3348 (86.98%) corneas were determined to be suitable for transplant. For the cause of death in 2009 and subsequent years, the incidence of trauma was lower (P = .001) compared with the years previous to 2009, whereas incidence of cardiac pathology (P = .014) was higher. The number of donors older than 50 years was higher for 2009 and years thereafter, compared with data from earlier years (P = .024). The rate of discarded corneas for unsuitable tissue morphology (P = .004) and total discarded corneas (P = .027) decreased in 2009 and years thereafter. The rate of discarded corneas for HIV was higher after for 2009 and years thereafter (P = .047), whereas the rate of discarded corneas for syphilis was significantly higher for 2015 and years thereafter (P = .001). CONCLUSIONS This study evaluated the most extensive eye bank data in Turkey and found that advances in lamellar surgery techniques and improvements in specular microscopic facilities have expanded the pool for cornealtransplants to include tissues from older donors. Moreover, the incidence rates for HIVpositive and syphilis-positive serology tests in discarded corneas have increased over time.

A rare case of hypothyroid coma developed in a patient with diagnosed primary hypothyroidism, complicated by the development of rhabdomyolysis and prolonged oppression of the respiratory center is presented. The predominance of previous cardiovascular pathology, marked oedema syndrome, and hypercholesterolemia in the clinical picture of the disease initially directed the diagnostics of cardiac pathology, which delayed the diagnosis of hypothyroidism. This publication aims to draw clinicians' attention to hypothyroid coma as a rare complication of hypothyroidism, which in this case was disguised as ischemic heart disease with the development of chronic heart failure.Представлено редкое наблюдение гипотиреоидной комы, развившейся у пациентки с недиагностированным первичным гипотиреозом, осложнившимся развитием рабдомиолиза и длительного угнетения деятельности дыхательного центра. Доминирование в клинической картине заболевания предшествующей патологии сердечно-сосудистой системы, выраженного отечного синдрома и гиперхолестеринемии первоначально направило диагностический поиск в сторону кардиальной патологии, что замедлило постановку диагноза гипотиреоза. Цель настоящей публикации привлечь внимание клиницистов к гипотиреоидной коме как редко встречающемуся осложнению гипотиреоза, протекавшему в данном случае под маской ишемической болезни сердца с развитием хронической сердечной недостаточности.

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.

Background During the last years, the concept of frailty has become increasingly familiar in many surgical specialties, including cardiac surgery. One of the main characteristics of frailty is its potentially reversible nature. With the aim of reducing the incidence of complications after surgery, efforts are being made to preoperatively decrease the level of frailty, however, the effect that the surgery itself may have in the postoperative frailty status is still unknown. Methods A prospective cohort of 137 patients aged 70 or more undergoing cardiac surgery with the use of cardiopulmonary bypass were recruited at three university-affiliated hospitals in Spain. Frailty status was assessed preoperatively and six months after surgery using two different validated frailty measurements: The Fried frailty scale and the Clinical Frailty Scale. We analyzed the changes in these frailty scores and the transitions between frailty status after surgery. Results Prevalence of frailty was 27.5% with the Fried Frailty Scale and 11% with the Clinical Frailty Scale. Even though frail patients presented a higher incidence of postoperative major complications than their robust counterparts, up to 50% of frail patients improved their frailty status after surgery. The distribution of frailty groups at baseline and six months after surgery changed significantly, decreasing the overall burden of frailty after surgery. Besides, robust patients showed a slightly worsening of their previous frailty scores. Conclusions Frailty is a multifactorial and dynamic condition that can be significantly reduced after cardiac surgery, suggesting that cardiac pathology is an important contributor to the preoperative patient’s frailty status.

Submitral left ventricular aneurysm is a rare cardiac pathology with very few cases reported in the literature. These are nonischemic aneurysms mostly reported from Africa. Patients with submitral aneurysm exhibit varied clinical manifestations. We report a case of calcified submitral aneurysm and its successful surgical management through a transaneurysmal approach.

Hospital readmission following transcatheter aortic valve replacement (TAVR) contributes considerably to the costs of care. Readmission rates following TAVR have been reported to be as high as 17.4% at 30 days and 53.2% at 1 year. Patient and procedural factors predict an increased likelihood of readmission including non-transfemoral access, acute and chronic kidney impairment, chronic lung disease, left ventricular systolic dysfunction, atrial fibrillation, major bleeding and prolonged index hospitalisation. Recent studies have also found the requirement for new pacemaker implantation and the severity of paravalvular aortic regurgitation and tricuspid regurgitation to be novel predictors of readmission. Post-TAVR readmission within 30 days of discharge is more likely to occur for non-cardiac than cardiac pathology, although readmission for cardiac causes, especially heart failure, predicts higher mortality than readmission for non-cardiac causes. To combat the risk of readmission and associated mortality, the routine practice of calculating and considering readmission risk should be adopted by the heart team. Furthermore, because most readmissions following TAVR occur for non-cardiac reasons, more holistic approaches to readmission prevention are necessary. Familiarity with the most common predictors and causes of readmission should guide the development of initiatives to address these conditions proactively.

In the absence of cardiac pathology, the presence of a dilated inferior vena cava (IVC) is considered idiopathic. To date, this phenomenon has only been described in athletic individuals as an adaptation to chronically augmented venous return. This is the largest prospective cohort study, following ten individuals with idiopathic dilated IVC against an age-matched control group with annual echocardiograms and cardiac magnetic resonance (CMR) imaging for a median of 55 months. No significant difference was found between echocardiography and CMR measurements in IVC diameter assessment both at baseline and at follow-up. Over the study period, there was no significant progression of the IVC in diameter as measured either by echocardiography or CMR. None of the patients suffered any cardiovascular events, and there were no hospitalizations. Our findings indicate the benign nature of this condition and provide reassurance with regard to future clinical implications.

Overlapping clinical features promoted the discussion of whether Kawasaki disease (KD) and PIMS-TS share pathophysiological features and disease outcomes. Medical records from English patients with KD (2015-02/20, N = 27) and PIMS-TS (02/2020-21, N = 34) were accessed to extract information. Children with PIMS-TS were older and more frequently of minority ethnicity background. They patients more commonly exhibited cytopenias and hyperferritinemia, which associated with diffuse cardiac involvement and functional impairment. In some PIMS-TS cases, cardiac pathology developed late, but outcomes were more favorable. In both, KD and PIMS-TS, baseline coronary diameter was a predictor of outcomes. PIMS-TS treatment more frequently included respiratory and cardiovascular support, and corticosteroids with IVIG. Cardiac involvement in PIMS-TS may be the result of a cytokine storm. Though more severe and diffuse when compared to KD, cardiac involvement of PIMS-TS has a more favorable prognosis, which may, after recovery, mitigate the need for long-term follow up.

Background and ObjectiveTricuspid regurgitation is a prevalent and undertreated cardiac pathology impacting millions across the globe. While historically surgical interventions for isolated tricuspid regurgitation were largely avoided due to data citing poor surgical outcomes, advances in transcatheter techniques and imaging modalities have reframed the approach to tricuspid valve disease in promising new ways.MethodsHere we sought to provide a landscape review of the current state of the field for transcatheter tricuspid valve interventions. We first start with a descriptive overview of the tricuspid valve, reviewing the anatomy, imaging characteristics, and the current guidelines for tricuspid interventions. We then review both transcatheter valve repair and valve replacement modalities, highlighting the devices, techniques, and valves currently under investigation, summarizing available outcomes data for each modality when possible.Results and ConclusionOur aim in writing this landscape review is the create an all-encompassing, up-to-date resource for clinicians to refer to when seeking to learn about the current state of transcatheter tricuspid valve interventions. We also hope to highlight the exciting promise of transcatheter tricuspid valve replacement in appropriate patients, and review the valves currently under development for use in the tricuspid position.

The SARS-COV2 Pandemic has required the emergency departments to focus their attention and care to COVID-19-diagnosed patients over patients with other pathologies. Descriptive study of patients attended in the resuscitation room between 1st of March and 31st May 2019 and compared to the same period in 2020. We include all the patients attended were included in the study and their clinical variables evaluated and their diagnosis at discharge. Six hundred and fifty-nine patients were attended in 2019 and 384 in 2020. There were no differences between age and gender. In 2019, 83.2% of the cases attended had a cardiac pathology, followed by neurological pathology and traffic accidents. This data is also significant since in the same period of 2020 cardiac pathology fell to 8.3%. The COVID pandemic has reduced patients attended at resuscitation room, and especially cardiovascular ones. These are preliminary results and more studies should be done to confirm or to study this trend.

Background Lung cancer screening (LCS) is broadly accepted. Screening also identifies incidental cardiac findings (S findings) that need follow-up. We report the magnitude of the potential downstream revenue generated by appropriate S finding management after 4 years of our free LCS program. Materials and Methods A retrospective database and chart review of a single-center free LCS program in the underserved southeast were performed. All patients who were enrolled in the screening required a primary care physician (PCP) as part of the decision-making model. Referrals to cardiac specialists for S findings found on LCS were recorded. Cost analysis was performed to track potential downstream revenue generated for the institution based upon Medicare allowable or Diagnosis-related group calculations. Results One thousand one hundred thirty-two scans were reviewed with 262 (23%) yielding positive S findings for 1 or more organ systems. 181/262 (69%) patients had cardiac findings, only 64/181 (35%) of these patients were referred to cardiology specialists by the PCP. The total Medicare billable amount for all cardiac referrals/interventions was $284 379, representing 35% of the potential billable amount of $804 260. Percutaneous coronary intervention (PCI) was the highest billable amount at $18 568. Eight percent of the patients undergoing appropriate cardiac evaluation required a PCI. If not for the screening and cardiac specialist referral, this patient group may not have received appropriate cardiovascular diagnosis and treatment. Discussion Lung cancer screening also identifies patients with significant cardiac disease, many of whom may not be appropriately referred. Identification and treatment of incidentally noted cardiovascular findings may both improve patient care and justify supporting free LCS programs.

BACKGROUND: Accurate visualization of cardiac valves and lesions by three-dimensional (3D) echocardiography is integral for optimal guidance of structural procedures and appropriate selection of closure devices. A new 3D rendering tool known as transillumination (TI), which integrates a virtual light source into the data set, was recently reported to effectively enhance depth perception and orifice definition. We hypothesized that adding the ability to adjust transparency to this tool would result in improved visualization and delineation of anatomy and pathology and improved localization of regurgitant jets compared with TI without transparency and standard 3D rendering. METHODS: We prospectively studied 30 patients with a spectrum of structural heart disease who underwent 3D transesophageal imaging (EPIQ system, Philips) with standard acquisition and TI with and without the transparency feature. Six experienced cardiologists and sonographers were shown randomized images of all three display types in a blinded fashion. Each image was scored independently by all experts using a Likert scale from 1 to 5, while assessing each of the following aspects: (1) ability to recognize anatomy, (2) ability to identify pathology, including regurgitant jet origin, (3) depth perception, and (4) quality of border delineation. RESULTS: TI images with transparency were successfully obtained in all cases. All experts perceived an incremental value of the transparency mode, compared with TI without transparency and standard 3D rendering, in terms of ability to recognize anatomy (respective scores: 4.5 ± 1.1 vs 4.1 ± 1.1 vs 3.6 ± 1.1, P < .05), ability to identify pathology (4.1 ± 1.1 vs 3.9 ± 1.2 vs 3.3 ± 1, P < .05), depth perception (4.6 ± 0.7 vs 4.1 ± 0.8 vs 3.2 ± 1.0, P < .05), and border delineation (4.6 ± 0.8 vs 4.1 ± 1.0 vs 3.1 ± 1.1, P < .05). CONCLUSIONS: The addition of the transparency mode to TI rendering significantly improves the diagnostic and clinical utility of 3D echocardiography and has the potential to markedly enhance echocardiographic guidance of cardiac structural interventions. (J Am Soc Echocardiogr 2020;33:1316–23.)

Background Troponin levels are frequently obtained in pediatric patients, but the benefit remains unclear. Methods and Results This retrospective study included 1993 patients aged 0 to 21 years without history of cardiac disease in whom troponin levels were obtained during clinical evaluation of cardiac and noncardiac presentations. Troponin was elevated (≥0.1 ng/mL) in 182 patients (9%). A cardiac diagnosis was made in 109 (60%) of those with elevated troponin and in 208 (12%) of those without ( P Conclusions Troponin can be a useful adjunctive test in the evaluation of children when the differential diagnosis includes cardiac etiologies. Serial measurement was not helpful when troponin was elevated at presentation but may merit consideration when the initial level is not elevated and there is ongoing concern about cardiac involvement. Lower reference values may be appropriate when evaluating children in contrast to adults.

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.

ObjectiveHistorically, electrocardiographic (ECG) interpretation criteria for athletes were only applicable to adults. New international recommendations now account for athletes ≤16 years, but their clinical appropriateness is unknown. We sought to establish the diagnostic accuracy of new international ECG recommendations against the Seattle criteria and 2010 European Society of Cardiology (ESC) recommendations in paediatric athletes using receiver operator curve analysis. Clinical context was calculated using Bayesian analysis.Methods876 Arab and 428 black male paediatric athletes (11–18 years) were evaluated by medical questionnaire, physical examination, ECG and echocardiographic assessment. ECGs were retrospectively analysed according to the three criteria.ResultsThirteen (1.0%) athletes were diagnosed with cardiac pathology that may predispose to sudden cardiac arrest/death (SCA/D) (8 (0.9%) Arab and (5 (1.2%) black)). Diagnostic accuracy was poor (0.68, 95% CI 0.54 to 0.82) for 2010 ESC recommendations, fair (0.70, 95% CI 0.54 to 0.85) for Seattle criteria and fair (0.77, 95% CI 0.61 to 0.93) for international recommendations. False-positive rates were 41.0% for 2010 ESC recommendations, 21.8% for Seattle criteria and 6.8% for international recommendations. International recommendations provided a positive (+LR) and negative (−LR) post-test likelihood ratio of 9.0 (95% CI 5.1 to 13.1) and 0.4 (95% CI 0.2 to 0.7), respectively.ConclusionIn Arab and black male paediatric athletes, new international recommendations outperform both the Seattle criteria and 2010 ESC recommendations, reducing false positive rates, while yielding a ‘fair’ diagnostic accuracy for cardiac pathology that may predispose to SCA/D. In clinical context, the ‘chance’ of detecting cardiac pathology within a paediatric male athlete with a positive ECG (+LR=9.0) was 8.3%, whereas a negative ECG (−LR=0.4) was 0.4%.

Cardiovascular diseases are among the most significant causes of mortality in humans. Pesticides toxicity and risk for human health are controlled at a European level through a well-developed regulatory network, but cardiotoxicity is not described as a separate hazard class. Specific classification criteria should be developed within the frame of Regulation (EC) No 1272/2008 in order to classify chemicals as cardiotoxic, if applicable to avoid long-term cardiovascular complications. The aim of this study was to review the cardiac pathology and function impairment due to exposure to pesticides (i.e. organophosphates, organothiophisphates, organochlorines, carbamates, pyrethroids, dipyridyl herbicides, triazoles, triazines) based on both animal and human data. The majority of human data on cardiotoxicity of pesticides come from poisoning cases and epidemiological data. Several cardiovascular complications have been reported in animal models including electrocardiogram abnormalities, myocardial infarction, impaired systolic and diastolic performance, functional remodeling and histopathological findings, such as haemorrhage, vacuolisation, signs of apoptosis and degeneration.

Endocardial endothelial cells (EECs) constitute an important component of the heart. These cells form a monolayer that covers the cavities of the right (EECRs) and left (EECLs) ventricles. They play an important role in cardiac excitation–contraction coupling via their secretion of cardioactive factors such as neuropeptide Y (NPY). They also contribute to cardiac pathology such as arrhythmia, hypertrophy, and heart failure. Differences between EECRs and EECLs contribute to tuning of circulating factors at the entry and exit of the ventricles. NPY, via activation of its receptors, modulates the excitation–secretion coupling of EECs, thus, indirectly modulating cardiac function and remodeling.

This commentary will provide a brief synopsis of the progress made in prediction and prevention of sudden cardiac death (SCD), the challenges that remain, and the opportunities available to make a real impact in this field. The dawning of the new millennium saw the prophylactic implantable defibrillator (ICD) firmly established as the major primary prevention modality, poised to make a major impact on the burden of SCD. More than a decade and a half later, has this expectation been realized? The modest impact of the primary prevention ICD on SCD burden is largely due to the now well-recognized inadequate performance of the left ventricular ejection fraction as a risk stratification tool. Consequently, the field has transitioned from a focus on the "high-risk ejection fraction" to the broader concept of the "high-risk patient". There are currently no effective means of stratifying SCD risk in patients with preserved EF, who constitute the majority (at least 70%) of all patients who will suffer SCD. Can the field be disrupted and novel predictors of SCD identified? In addition to the ongoing quest for identification of the high-risk patient early in the nature history of SCD, a new paradigm for preventing SCD in the "near-term", within several weeks of the lethal event, has been proposed. While rapid advances in technology, data warehousing and analysis will accelerate the process of enhancing SCD prediction and prevention; regulatory, funding and clinical implementation strategies will need to keep pace if these expectations are to be realized.

The cardiovascular benefits of habitual exercise are well documented. In the current era, more of the population is exceeding the recommendations for physical activity as the popularity of endurance events increases. Recent data have proposed a U-shaped relationship between exercise intensity and cardiovascular outcomes. Regular participation in endurance activities has been shown to result in structural and functional changes in the heart. This re-modelling may be the substrate for cardiac dysfunction or arrhythmias. The risk of sudden cardiac death may also be elevated; however, in most cases of sudden cardiac death, the cause can be linked to an underlying cardiac pathology where exercise acted as the trigger for a lethal arrhythmia. This article serves to review whether excessive exercise may result in harm in some athletes.

Background Aortic coarctation is a hereditary disease often accompanied by ascending aortic aneurysm and aortic valve disease. We introduce a single-stage surgical treatment option for patients with aortic coarctation, ascending aortic aneurysm, and aortic valve disease, with a comparative analysis of the results. Methods The study included six patients with aortic coarctation, ascending aortic aneurysm, and aortic valve disease. All operations were performed by a full median sternotomy. For a comparative analysis, the patients were divided into two groups: 3 patients underwent ascending-to-descending aortic bypass, and the other 3 underwent surgical aortic coarctation repair. The short- and medium-term postoperative periods were monitored. The mean duration of monitoring was 27 months. Results When comparing the 2 groups, we observed a tendency for improvement in all intraoperative parameters in the 2nd group. There was no mortality in the postoperative hospital period. One (16.7%) patient died of stroke in the medium-term postoperative period. The other 5 patents showed no signs of procedure-related complications. Conclusion We recommend performing a single-stage procedure for surgical treatment of coarctation of the aorta with concomitant aneurysm of the ascending aorta and aortic valve disease. Compared to ascending-to-descending aortic bypass, surgical repair of aortic coarctation is more physiological, feasible via a sternotomy, and tends to improve intraoperative factors.

OBJECTIVES To: (1) analyze the results of 5 years of preparticipation cardiac screening including 12-lead electrocardiogram (ECG) of National Collegiate Athletic Association (NCAA) Division I athletes; and (2) assess the rates of ECG screening abnormalities and false-positive rates among 3 ECG screening criteria. DESIGN Retrospective chart review. SETTING National Collegiate Athletic Association Division I University. PARTICIPANTS One thousand six hundred eighty-six first-year athletes presenting for their preparticipation examination including 12-lead resting ECG. INTERVENTIONS At the completion of the study period, all ECGs were retrospectively reviewed using the Seattle, Refined, and International Criteria. MAIN OUTCOME MEASURES (1) Prevalence of pathologic cardiac conditions identified by screening; and (2) number of ECG screening abnormalities by criteria. RESULTS Three athletes (0.2%) were found to have conditions that are associated with sudden cardiac death. Retrospective review of ECGs using Seattle, Refined, and International criteria revealed an abnormal ECG rate of 3.0%, 2.1%, and 1.8%, respectively. International criteria [odds ratios (OR), 0.58; P = 0.02] demonstrated a lower false-positive rate compared with the Seattle criteria. There was no significant difference in false-positive rates between the Seattle and Refined (OR, 0.68; P = 0.09) or the International and Refined criteria (OR, 0.85; P = 0.5). CONCLUSIONS There was a low rate of significant cardiac pathology in this population, and no athletes were permanently restricted from play as a result of screening. Our results suggest that the International criteria have the lowest false-positive rate of athlete-specific ECG criteria, and thus, it is the preferred method for preparticipation ECG screening in NCAA athletes.

AIMS: Arrhythmogenic Cardiomyopathy (AC) is an inherited, frequently under diagnosed disorder, predisposing to sudden cardiac death. Rare, recessive forms of AC can be associated with woolly hair and palmoplantar keratoderma, but most autosomal dominant AC forms have been reported as cardiac specific. Causative mutations frequently occur in desmosomal genes including desmoplakin (DSP) In this study, we have systematically investigated the presence of a skin and hair phenotype in heterozygous desmoplakin (DSP) mutation carriers with AC. METHODS AND RESULTS: 6 AC pedigrees with 38 carriers of a dominant loss of function (nonsense or frameshift) mutation in DSP were evaluated by detailed clinical examination (cardiac, hair and skin) and molecular phenotyping. All carriers with mutations affecting both major DSP isoforms (DSP I and II) were observed to have curly or wavy hair in the pedigrees examined except for members of Family 6, where the position of the mutation only affected the cardiac-specific isoform, DSP I. A mild palmoplantar keratoderma was also present in many carriers. Sanger sequencing of cDNA from non-lesional carrier skin suggested degradation of the mutant allele. Immunohistochemistry of patient skin demonstrated mislocalisation of DSP and other junctional proteins (plakoglobin, connexin 43) in the basal epidermis. However, in Family 6, DSP localisation was comparable to control skin. CONCLUSIONS: This study identifies a highly recognizable cutaneous phenotype associated with dominant, loss of function DSPI/II mutations underlying AC. Increased awareness of this phenotype amongst healthcare workers could facilitate a timely diagnosis of AC in the absence of overt cardiac features.

The reported causes of sudden death (SD) in young people at autopsy include a variety of structural, often genetic, heart diseases. In young competitive athletes, arrhythmogenic cardiomyopathy (AC) in Italy [(1)][1] and hypertrophic cardiomyopathy (HCM) in the United States [(2)][2] are the most

Human cardiac drug discovery and disease modeling face challenges in recapitulating cellular complexity and animal-to-human translation due to the limitations of conventional 2D cell culture and animal models. The development of human cardiac organoid technologies could help in stimulating and maintaining differentiated cell functions for extended periods of time. By closely mimicking in vivo organ functions in vitro they could thereby help in overcoming the obstacles mentioned above. Through the construction of human cardiac organoids from pluripotent stem cell-derived cells, derived from patients with specific known genotypes and phenotypes, more complex and robust in vitro tools have recently become available for disease modeling. In this review, we will describe the relevance and importance of evolving organoid platforms in disease biology. We further provide examples of cardiac organoid platforms, which may lead the way toward future personalized medicine and drug discovery.

International audience; 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 seg-mentation 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 explain-able, 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.

From detailed characterization of cardiac abnormalities to the assessment of cancer treatment-related cardiac dysfunction, cardiac MRI is playing a growing role in the evaluation of cardiac pathology in oncology patients. Current guidelines are now incorporating the use of MRI for the comprehensive multidisciplinary approach to cancer management, and innovative applications of MRI in research are expanding its potential to provide a powerful noninvasive tool in the arsenal against cancer. This review focuses on the application of cardiac MRI to diagnose and manage cardiovascular complications related to cancer and its treatment. Following an introduction to current cardiac MRI methods and principles, this review is divided into two sections: functional cardiovascular analysis and anatomical or tissue characterization related to cancer and cancer therapeutics. Level of Evidence: 5 Technical Efficacy Stage: 1 J. Magn. Reson. Imaging 2019;50:1349-1366.

Medical care in Canadian cardiac units has changed considerably over the past 3 decades in response to an increasingly complex and diverse patient population admitted with acute cardiac pathology. To maintain the highest level of care for these patients, there is a pressing need to evolve traditional coronary care units into contemporary cardiac intensive care units. In this article we aim to highlight the current variations in Canadian units, develop approaches to overcome logistical and infrastructural obstacles, and propose staffing and training recommendations that would allow for the establishment of contemporary cardiac intensive care units.

Exercise-induced cardiac remodelling (EICR) refers to the cardiac structural and functional adaptations that occur in response to the hemodynamic stress of strenuous exercise. Differentiating physiological cardiac hypertrophy as a result of EICR from structural cardiac pathology may be challenging in clinical practice because of the phenotypic crossover between extreme forms of the "hearts of athletes" and mild forms of cardiomyopathy. This structural phenotypic overlap equates to a grey zone of clinical uncertainty. Specifically, asymptomatic athletes presenting with extreme left ventricular (LV) dilatation, LV wall thickening, or right ventricular (RV) dilatation require a systematic and integrative diagnostic approach to achieve accurate clinical differentiation. The combination of a careful clinical history and examination, appropriately used multimodality cardiac imaging, functional exercise testing, ambulatory rhythm monitoring, and occasional detraining typically provides the necessary data for diagnostic purposes and sports participation recommendations. Further clinical distinction of the hearts of athletes from cardiac pathology may emerge from future clinical and translational research efforts establishing exercise-related biomarker profiles and mechanisms underlying EICR adaptations.

Diseases of the cardiac conduction system can be debilitating and deadly. Electronic pacemakers are incredibly effective in the treatment of sinus and AV node dysfunction, yet there remain important limitations and complications. These issues have driven interest in the development of a biological pacemaker. Here, we review experimental progress in animal models and discuss future directions, with a focus on reprogramming endogenous cells in the heart to treat defects of rhythm and conduction.

The idea of isolated organ perfusion, a precursor of cardiopulmonary bypass, came by Legalois in 1812. First isolated organ perfusion was described by Loebell in 1849. The first closed system for oxygenation and returning the blood through arteries was created by Frey and Gruber in 1885. Gibbon Jr. is considered the father of extracorporeal circulation. In spring of 1934 he began constructing a machine for extracorporeal circulation in Boston. He published the first description of this system in 1937. Gibbon won the grant of the International Business Machines Corporation for developing the machine in 1947. Together they developed Model I in 1949 and Model II in 1951. After a few unsuccessful attempts in 1952, the first successful surgical intervention on the heart (closure of atrial septal defect) using cardiopulmonary bypass was performed on May 6, 1953. In 1945, Kirklin and his working group reported on a series of eight successfully treated patients in a row who underwent surgery with extracorporeal circulation. First successful valve surgery under the direct vision was performed by Dodrill in 1952, using his ?Michigan Heart? machine as a right heart bypass. Using cardiopulmonary bypass, cardiac surgeons can deal with the complex cardiac pathology and save millions of lives.

The neuronal ceroid lipofuscinoses (NCLs) are mostly seen as diseases affecting the central nervous system, but there is accumulating evidence that they have co-morbidities outside the brain. One of these co-morbidities is a decline in cardiac function. This is becoming increasingly recognised in teenagers and adolescents with juvenile CLN3, but it may also occur in individuals with other NCLs. The purpose of this review is to summarise the current knowledge of the structural and functional changes found in the hearts of animal models and people diagnosed with NCL. In addition, we present evidence of structural changes that were observed in a systematic comparison of the cardiomyocytes from CLN3Δex7/8 mice.

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.