Introduction . The diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) remains a challenging task. Despite the revised 2010 criteria of the ITF Task Force, most patients still face difficulties in establishing this diagnosis. Objective . To assess the clinical and genetic characteristics of patients with a preliminary diagnosis of “ARVC,” with a focus on the final diagnosis established after re-evaluation at the Republican Scientific and Practical Center of Cardiology (RSPC “Cardiology”). Material and methods . Twenty-eight patients (53.6% male, median age 35 [24; 43] years) with a preliminary diagnosis of ARVC referred to the RSPC “Cardiology” were examined. The clinical and instrumental evaluation included 12-lead ECG, 24-hour Holter monitoring, transthoracic echocardiography (TTE), and cardiac magnetic resonance imaging (MRI) with late gadolinium enhancement (LGE). The ARVC diagnosis was based on the 2010 ITF criteria and classified into definite, borderline, and possible categories. Genetic testing for mutations in genes associated with cardiomyopathies and inherited rhythm disorders was performed using next-generation sequencing (NGS). Results . Among the 28 patients with a preliminary diagnosis of ARVC, only 7 (25%) were diagnosed with definite ARVC according to the 2010 ITF criteria and confirmed by genotyping (mutations in the PKP2 and DSG genes in 5 patients, mutation in the non-desmosomal TMEM43 gene in 1 patient, and mutations in ILK and RBM20 genes in 1 patient). Eight (28.6%) patients were diagnosed with borderline ARVC, and 4 (14.3%) with possible ARVC. In 9 (32.1%) patients, the diagnosis of ARVC was ruled out due to misinterpretation of cardiac MRI. Genotyping revealed no ARVC-associated mutations in the borderline, possible, or excluded groups. A total of 7 (25%) patients received an ICD; of these, 6 were deemed appropriate, while 1 was considered inappropriate due to an unconfirmed ARVC diagnosis. Conclusions . A misdiagnosis of ARVC was found in 32.1% of patients suspected of having the condition. The most common reason for false suspicion was misinterpretation of cardiac MRI results, particularly regional dyskinesia/akinesia of the right ventricular wall due to factors such as pericardial constriction, right ventricular diverticulum, pectus excavatum, right ventricular enlargement in athletes, or frequent arrhythmias causing artifacts. Genotyping proved helpful in timely confirmation of the ARVC diagnosis in this patient cohort.

Return-to-play in an endurance athlete with recurrent pericarditis and transient pericardial constriction: A case report

Risk Factors for Pericardial Constriction

In healthcare, scarcity of high-quality human-adjudicated labelled data may limit the potential of deep neural networks (DNNs). Foundation models provide an efficient starting point for deep learning that can facilitate effective DNN training with fewer labelled training examples. In this study, we leveraged cardiologist-confirmed labels from a large dataset of 1.6 million electrocardiograms (ECGs) acquired as part of routine clinical care at UCSF between 1986 and 2019 to pre-train a convolutional DNN to predict 68 common ECG diagnoses. To our knowledge, this model is one of the most comprehensive ECG DNN models to date, demonstrating high performance with a median area under the receiver operating curve (AUC) of 0.978, median sensitivity of 0.937, and median specificity of 0.923. We then demonstrate the model's utility as a foundation model by additionally training (fine-tuning) the DNN to detect three novel ECG diagnoses with relatively small datasets: carcinoid syndrome, pericardial constriction, and rheumatic doming of the mitral valve. Fine-tuning training of the foundation model achieved an AUC of 0.772 (95% CI 0.723-0.816) for carcinoid syndrome, 0.883 (0.863-0.906) for pericardial constriction, and 0.826 (95% CI 0.802-0.854) for rheumatic doming, compared to 0.492 (95% CI 0.434-0.558), 0.689 (95% CI 0.656-0.720), and 0.701 (95% CI 0.657-0.745), respectively, for DNNs trained from scratch on the same small datasets. Our results demonstrate that the ECG foundation model learned a flexible representation of ECG waveforms and can improve performance of fine-tuned downstream models, particularly in data-limited settings.

Aim: To present a case of ‘end-stage’ constrictive pericarditis with clinical manifestations such as ascites mimicking as cirrhosis of liver. Introduction: ‘End-stage’ constrictive pericarditis has been readily confused with cirrhosis of liver in which there may be ascites, but venous pressure is normal and the neck veins are not engorged. There may be cardiac enlargement in other causes of heart failure. Etiology remains unknown in majority of case and inflammatory process play a central role in its development. Case Report: A 67-year-old male presented with sudden onset of tachycardia. Clinical examination revealed right-sided heart failure, ‘Egg-shell’ calcification in Chest X-ray and a characteristic echocardiographic feature of pericardial constriction such as septal bounce and dynamic respiratory changes in mitral inflow velocity. The patient was advised medical measures since it is in advanced stage. Conclusion: When clinical signs of right heart failure become unresponsive to increased doses of diuretics, constrictive pericarditis is more likely the underlying disease since severe, right-sided failure develops in very advanced, the end-stage of the disease.

Aim: To present a case of ‘end-stage’ constrictive pericarditis with clinical manifestations such as ascites mimicking as cirrhosis of liver. Introduction: ‘End-stage’ constrictive pericarditis has been readily confused with cirrhosis of liver in which there may be ascites, but venous pressure is normal and the neck veins are not engorged. There may be cardiac enlargement in other causes of heart failure. Etiology remains unknown in majority of case and inflammatory process play a central role in its development. Case Report: A 67-year old male presented with sudden onset of tachycardia. Clinical examination revealed right-sided heart failure, ‘Egg-shell’ calcification in Chest X-ray and a characteristic echocardiographicfeatures of pericardial constriction such as septal bounce and dynamic respiratory changes in mitral inflow velocity. The patient was advised medical measures since it is in advanced stage. Conclusion: When clinical signs of right heart failure become unresponsive to increased doses of diuretics, constrictive pericarditis is more likely the underlying disease since severe, right-sided failure develops in very advanced, the end-stage of the disease.

Introduction: The objectives of this study were to determine the frequency of tuberculous pericarditis and to describe its diagnostic, therapeutic, and prognostic aspects. Methodology: We conducted a retrospective descriptive and analytical study over five years from January 1, 2015, to December 31, 2019, at the General Hospital Idrissa POUYE in Dakar. The study focused on patients aged over 18 years who were hospitalized for tuberculous pericarditis, with or without effusion, and treated with antituberculous drugs according to the protocol established by the National Tuberculosis Control Program (PNLT) of Senegal. This work was carried out in collaboration with the hospital management after obtaining approval from the cardiology department by signing the access form to patient records. Data were entered using Sphinx software version 5.1.0.2 and analyzed with SPSS (Statistical Package for Social Sciences) software version 18. Results: We identified 108 cases of pericarditis from all causes, representing a hospital prevalence of 1.95%. Among these, 42 were of tuberculous origin, with a hospital prevalence of 0.76% and 38.89% of all pericarditis cases, with a male predominance (sex ratio of 1.33). The mean age was 28.6 ± 10 years, with a predominance in the 20-29 age group (57.1%). More than half of the patients (57.1%) had a low socio-economic status, and 42.85% had a history of contact with tuberculosis. Symptoms were mainly dyspnea and chest pain (78.57% each). Tachycardia (85.7%) and pericardial friction rub (42.9%) were frequently observed. The pericardial puncture fluid was sero-hematic in 66.7%, and the study showed a positive Rivalta test with lymphocytic predominance. The adenosine deaminase (ADA) level in the pericardial puncture fluid, performed in 12.5% of patients, was positive. HIV serology was positive in 10% of cases. Cardiac ultrasound showed effusion in all patients, with a large amount in 64.29% of cases. All patients received antituberculous treatment, with 35.7% also receiving corticosteroids. The average hospital stay was 11 days, with complication rates of pericardial constriction (14.3%) and deaths (7.1%). Conclusion: Tuberculous pericarditis remains a significant health issue, particularly among patients of low socio-economic status and those with a history of contact with tuberculosis. Despite the availability of treatments, complication and mortality rates remain considerable, highlighting the need for early diagnosis and comprehensive management.

Introduction. Constrictive pericarditis is a long-term consequence of any pathological process developing in the pericardium due to fibrinous thickening and calcification of its layers, which prevents normal cardiac diastolic filling. One of its forms is transient constrictive pericarditis, which resolves after anti-inflammatory therapy.Brief description. A 19-year-old man with Bruton disease was admitted to the clinic with complaints of severe weakness, shortness of breath at rest, cough, chest pain, hyperthermia to 380 C, decreased blood pressure to 80/60 mm Hg, and abdominal distension. During the initial echocardiography, the results did not raise serious suspicions. However, given the rather specific interventricular septum motion, pericardial constriction was suspected.Discussion. During the additional examination, echocardiography made it possible to verify constrictive pericarditis, which was subsequently confirmed by heart catheterization. Due to significant immunoglobulin level deviations, a decision was made to resume immunoglobulin replacement therapy, followed by a clinical status reassessment.The article presents a clinical description of a patient with constrictive pericarditis, which was a manifestation of Bruton disease. During follow-up, resolution of constriction was noted with resumption of immunoglobulin replacement therapy.

Tuberculosis (TB) and human immunodeficiency virus/acquired immunodeficiency syndrome have reached epidemic proportions, particularly affecting vulnerable populations in low- and middle-income countries of sub-Saharan Africa. TB pericarditis is the commonest cardiac manifestation of TB and is the leading cause of constrictive pericarditis, a reversible (by surgical pericardiectomy) cause of diastolic heart failure in endemic areas. Unpacking the complex mechanisms underpinning constrictive haemodynamics in TB pericarditis has proven challenging, leaving various basic and clinical research questions unanswered. Subsequently, risk stratification strategies for constrictive outcomes have remained unsatisfactory. Unique pericardial tissue characteristics, as identified on cardiovascular magnetic resonance imaging, enable us to stage and quantify pericardial inflammation and may assist in identifying patients at higher risk of tissue remodelling and pericardial constriction, as well as predict the degree of disease reversibility, tailor medical therapy, and determine the ideal timing for surgical pericardiectomy.

Tuberculous (TB) pericarditis (TBP), a TB of the heart, is linked to significant morbidity and mortality rates. Administering glucocorticoid therapy to individuals with TBP might enhance overall results and lower the likelihood of fatality. However, the actual clinical effectiveness of supplementary glucocorticoids remains uncertain. This study specifically evaluated the effects of prednisolone, prednisolone-antiretroviral therapy (ART) interaction, and other potential risk factors in reducing the hazard of the composite outcome, death, cardiac tamponade, and constriction, among TBP and human immunodeficiency virus (HIV) patients. The data used in this study were obtained from the investigationof the Management of Pericarditis trial, a multicentre international randomized double-blind placebo-controlled factorial study that investigated the effects of two TB treatments, prednisolone and Mycobacterium indicus pranii immunotherapy in patients with TBPin Africa. This study used a sample size of 587 TBP and HIV-positivepatients randomized into prednisolone and its corresponding placebo arm. We used the extended Cox-proportional hazard model to evaluate the effects of the covariates on the hazard of the survival outcomes. Models fitting and parameter estimation were carried out using R version 4.3.1. Prednisolone reduces the hazard of composite outcome (hazrad ratio [HR] = 0.32, 95% confidence interval [CI] = , p < 0.001), cardiac tamponade (HR = 0.14, 95%CI = 0.05, 0.42, p < 0.001) and constriction (HR = 0.81, 95%CI = 0.41, 1.61, p = 0.55). However, prednisolone increases the hazard of death (HR = 1.58, 95%CI = 1.11, 2.24, p = 0.01). Consistent usage of ART reduces the hazard of composite outcome, death, and constriction but insignificantly increased the hazard of cardiac tamponade. The study offers valuable insights into how prednisolone impact the hazard of different outcomes in patients with TBP and HIV. The findings hold potential clinical significance, particularly in guiding treatment decisions and devising strategies to enhance outcomes in this specific patient group. However, there are concerns about prednisolone potentially increasing the risk of death due to HIV-related death.

Hemodynamic Manifestations of Concomitant Radiation-Induced Tricuspid Regurgitation and Pericardial Constriction Undergoing Transcatheter Tricuspid Valve Repair.

Pericardial Infiltration and Constriction Due to Cardiac Actinomycosis: A Case Report

Diagnosis and surgical management of pericardial constriction after cardiac surgery

10 Years of Pericardiectomy at a Tertiary New Zealand Hospital: Clinical Characteristics and Outcomes

Primary pericardial mesothelioma (PM) is a rare tumor arising from the mesothelial cells of the pericardium. It has an incidence of <0.05% and comprises <2% of all mesotheliomas; however, it is the most common primary malignancy of the pericardium. PM should be distinguished from secondary involvement by the spread of pleural mesothelioma or metastases, which are more common. Although data are controversial, the association between asbestos exposure and PM is less documented than that with other mesotheliomas. Late clinical presentation is common. Symptoms may be nonspecific but are usually related to pericardial constriction or cardiac tamponade, and diagnosis can be challenging usually requiring multiple imaging modalities. Echocardiography, computed tomography, and cardiac magnetic resonance demonstrate heterogeneously enhancing thickened pericardium, usually encasing the heart, with findings of constrictive physiology. Tissue sampling is essential for diagnosis. Histologically, similar to mesotheliomas elsewhere in the body, PM is classified as epithelioid, sarcomatoid, or biphasic, with the biphasic type being the most common. Combined with morphologic assessment, the use of immunohistochemistry and other ancillary studies is helpful for distinguishing mesotheliomas from benign proliferative processes and other neoplastic processes. The prognosis of PM is poor with about 22% 1-year survival. Unfortunately, the rarity of PM poses limitations for comprehensive and prospective studies to gain further insight into the pathobiology, diagnosis, and treatment of PM.

Abstract Background Radiotherapy plays a key role in the multimodality treatment of thoracic tumors. Radiotherapy-induced heart disease (RIHD) has become an increasingly recognized adverse reaction contributing to major radiation-associated toxicities, including nonmalignant death. Especially patients with diseases with excellent prognosis, such as breast cancer or Hodgkin's lymphoma, may suffer from delayed side effects 2-6 including RIHD in a dose-dependent manner. The pathological spectrum of RIHD includes conduction abnormalities, valvular disease, coronary artery disease, pericarditis and pericardial constriction or effusion, cardiomyopathy, and myocardial fibrosis. Here we describe the case of a young man cured of Hodgkin's lymphoma who presented to our laboratory with the diagnosis of suspected myocarditis in the Sars-COV 2 era, but the presenting clinical picture confused the clinicians and complex coronary artery disease was behind it. Method-Clinical Case A young 33-years-old man presented to the emergency room with typical exertional chest pain. Clinical history: smoker patient who denied familiarity for cardiovascular diseases, dyslipidemic, 10 years previously underwent chemotherapy and radiotherapy for Hodgkin's Lymphoma in complete remission. A nasopharyngeal molecular swab for Sars-COV 2 was performed, which was negative. The presentation electrocardiogram (EKG) documented nonspecific repolarization abnormalities; the myocardionecrosis enzyme curve performed at three times was frankly positive with elevated PCR values ​​(102 pg/ml). Color Doppler echocardiography documented a left ventricular ejection fraction at the lower limits of normal, hypokinesia of the mid-basal segments of the infer-posterolateral wall with moderate mitral valve regurgitation. On suspicion of acute myocarditis, the patient was transferred to the Coronary Care Unit and, during admission, underwent MRI, which showed a slightly enlarged left ventricle (DTD 58 mm, EDV 147 ml), slightly depressed systolic function (LVEF 46%), akinesia of the proximal lateral and mid-proximal wall. In delayed enhancement sequences late persistence of gadolinium in the endomesocardium (60%), proximal lateral and mid-proximal wall with involvement of areas adjacent to the base of implantation of both papillary muscles. In light of the instrumental picture, the patient underwent coronarography, which showed an unexpected nightmare picture, given his young age. Circumflex branch (lcx-lesion culprit) suboccluded to the middle segment with TIMI I downstream flow at the bifurcation with a prominent obtuse marginal branch (OM) with a delayed reperfusion (Medina 1,1,1); diffusely atheromatous left anterior descending artery (LAD), showing 70% complex critical disease in the proximal segment at the bifurcation with a first diagonal branch of good caliber and good distribution area (Medina 1,1,1). Clinical resolution/Results Therefore, in a patient with misdiagnosed ACS-NSTEMI, two complex coronary bifurcation angioplasties according to TAP technique (Fig 3-4) were performed through left radial access with Slender 7 in 6 introducer at one time. The following drugs were administered in the cath-lab: Cangrelor bolus/kg followed by continuous infusion for 2 hours and Prasugrel 60 mg, initially UFH 5000 IU and anticoagulation control according to ACT during the procedure. The procedure ended with complete revascularization and asymptomatic patient. During the following days of hospitalization, no late electrical or mechanical complications occurred. Conclusions The one just described represents a complex and unexpected scenario for a young adult. The literature available has analyzed the pathophysiology of myocardial damage resulting from exposure to high amounts of radiation in patients undergoing curative radiotherapy for Hodgkin's lymphoma. It is now generally accepted that the most common clinical syndromes after irradiation are pericarditis in acute and chronic forms,. However, coronary vessel lesions have been considered exceptionally rare, so the true pathophysiological triggering mechanism is still poorly understood. The most widely accepted hypothesis on the onset of RICHD is a dual pathway of vascular damage ("two-hit combined hypothesis"). The most important preventive measure regarding RICHD is dose minimization. Few data are available in the literature on outcomes according to the revascularization strategy adopted in patients with RICHD (PCI vs. CABG). Morbidity and mortality from post-radiotherapy cardiovascular complications in patients with Hodgkin's lymphoma must be reduced through close cardiological surveillance in primary prevention and a close collaboration between oncologists and cardiologists in order to minimize any deleterious complications, especially in the young. Further research is needed to elucidate profibrotic mechanisms, identify promising therapies that can be implemented early during the course of treatment and to compare revascularization strategies with longer-term mortality in such patients, in order to guide the physicians in the decision-making.

Introduction: Acute pericarditis has not been linked to long-term heart-specific morbidity in breast cancer patients. Carcinomatous pericardial involvement has been detected at autopsy in 10-20% of breast cancer patients. In clinical practice, when it comes to primarily echocardiographically-diagnosed pericardial diseases: pericardial effusion, cardiac tamponade, and pericardial constriction have all been reported in breast cancer patients. Acute pericarditis is primarily a clinical non-imaging bedside diagnosis derived from the combination of chest pain, pericardial rub, and typical electrocardiographic changes, irrespective of the presence or absence of pericardial fluid on imaging. It can be the initial clinical manifestation of a malignancy. Methods: The Myocardial Infarction Data Acquisition System (MIDAS) database is an ongoing, longitudinal, and validated database that comprises discharge data, along with demographics, comorbidities, and length of hospital stay; for all patients with cardiovascular diseases admitted to every non-federal acute care hospital in the state of New Jersey. We searched for the diagnosis of acute pericarditis (ICD-9 codes 420.0, 420.90, 420.91, and 420.99), in first hospital admissions of female patients with breast cancer (ICD-9 codes 174.0-174.9) between January 1995 to December 2015. Controls were female breast cancer patients without the diagnosis of acute pericarditis. Results: There were 60,435 female patients with breast cancer. Of those, 253 (0.4%) were also diagnosed with acute pericarditis on the same admission as the first breast cancer diagnosis, or later. Analysis for comorbidities showed that 116 (45.8%) of the 253 patients with acute pericarditis had admissions for heart failure, as opposed to 26.4% (15,895 out of 60,182) of breast cancer patients without acute pericarditis (p &lt; 0.00001). Conclusions: Since New Jersey has a diverse population that resembles the profile of the United States in age, gender, and race/ethnicity; our findings could also be generalized to other geographic areas, and may help with future clinical guidelines. Although it is rare, acute pericarditis in breast cancer patients can indicate long-term cardiovascular morbidity.

Introduction We describe a case of severe cardiomyopathy complicated by pericardial constriction resulting from epicardial VT ablation requiring heart transplant listing. Case Presentation A 43-year-old with a history of silent MI, LV aneurysm and thrombus, cardiomyopathy (EF 15%), Stage C heart failure, and VT on amiodarone initially presented with symptomatic VT requiring AICD therapy. He underwent epicardial ablation with no immediate complications. Post-ablation, he developed polymorphic VT prompting LHC revealing new LAD artery stenosis treated with stents. He was discharged on DAPT and warfarin. He subsequently re-presented with worsening dyspnea, lethargy, and edema. Invasive hemodynamics revealed elevated filling pressures and cardiogenic shock with constrictive physiology. Milrinone and aggressive diuresis were initiated with mild improvement in symptoms. Echocardiogram and cardiac MRI confirmed diagnosis of constrictive pericarditis and presence of hemopericardium. Discussion Multidisciplinary discussions were held to determine treatment. Management of constrictive pericarditis with pericardial stripping was considered. The high-risk nature of the procedure with unknown benefit, his age, and now Stage D heart failure made sternotomy a potential increased risk for future cardiac transplant. Ultimately, decision was made for pericardiocentesis, treatment with colchicine, a switch from triple therapy to warfarin and aspirin, and listing for transplant. Surveillance RHC 6 weeks post hospitalization showed resolution of constrictive physiology and normal cardiac output on milrinone.

TAMPONADE WAS ONLY A FACADE: CANCER WAS THE REAL ANSWER

BackgroundThe American College of Cardiology Core Cardiovascular Training Statement (COCATS) defined echocardiography core competencies and set the minimum recommend number of echocardiograms to perform (150) and interpret (300) for independent practice in echocardiography (level 2 training). Fellows may lack exposure to key pathologies that are relatively infrequent, however, even when achieving an adequate number of studies performed and interpreted. We hypothesized that cardiology fellows would lack exposure to 1 or more cardiac pathologies related to core competencies in COCATS when performing and interpreting the minimum recommend number of studies for level 2 training.MethodsWe retrospectively reviewed 11,250 reports from consecutive echocardiograms interpreted (7,500) and performed (3,750) by 25 cardiology fellows at a University tertiary referral hospital who graduated between 2015 and 2019. The first 300 echocardiograms interpreted and the first 150 echocardiograms performed by each fellow were included in the analysis. Echocardiography reports were reviewed for cardiac pathologies relating to core competencies defined in COCATS.ResultsAll 25 fellows lacked exposure to 1 or more cardiac pathologies related to echocardiography core competencies despite meeting COCATS minimum recommended numbers for echocardiograms performed and interpreted. Pathologies for which 1 or more fellows encountered 0 cases despite meeting the minimum recommended numbers for both echocardiograms performed and interpreted included: pericardial constriction (16/25 fellows), aortic dissection (15/25 fellows), pericardial tamponade (4/25 fellows), valvular mass/thrombus (2/25 fellows), prosthetic valve dysfunction (1/25 fellows), and cardiac chamber mass/thrombus (1/25 fellows).ConclusionsCardiology fellows who completed the minimum recommend number of echocardiograms performed and interpreted for COCATS level 2 training frequently lacked exposure to cardiac pathologies, even in a University tertiary referral hospital setting. These data suggest that fellowship programs should monitor pathology case counts for each fellow in training, in addition to the minimum recommend number of echocardiograms defined by COCATS, to ensure competency for independent practice in echocardiography.