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Hypereosinophilic syndrome is a rare systemic condition characterized by eosinophil-mediated organ damage. Cardiac involvement is common and typically occurs in sequential stages. We present two cases that demonstrate these different stages and presentations of eosinophilia-mediated myocardial disease, where multimodality imaging was essential for the diagnosis. More importantly, they demonstrate, for the first time, the dissociation between the eosinophil count and patients' clinical evolution, suggesting the need for close follow up even after the eosinophilia has been controlled. Cardiac involvement in hypereosinophilic syndrome typically occurs in three stages – necrotic, thrombotic, and fibrotic. Although cardiac damage is mediated by eosinophils, the blood eosinophil count and patients' clinical evolution are dissociated. Therefore, eosinophil count on its own is not an adequate marker of clinical evolution, and cardiac follow up should be continued even after the eosinophilia has been controlled. [ABSTRACT FROM AUTHOR]

Background Acute coronary syndrome (ACS) represents a common cardiovascular disease that poses a significant threat to human health, with severe coronary artery lesions and myocardial fibrosis affecting patient prognosis. The use of non-invasive serological markers to assess the severity of coronary artery lesions and myocardial fibrosis can aid in the early risk stratification and management of ACS patients. Objective This study aimed to explore the relationship between serum levels of complement C1q/tumor necrosis factor-related protein-5 (CTRP5) and the severity of coronary artery lesions, as well as myocardial fibrosis markers in ACS patients, to provide guidance for the clinical assessment of coronary artery lesion severity and myocardial fibrosis. Methods A total of 184 patients with obstructive stenosis admitted to the Department of Cardiology, Hengshui People's Hospital from February 2021 to February 2022 were selected, along with 100 ACS patients without significant obstructive stenosis undergoing coronary angiography in the same period as controls. General patient data were collected, Doppler echocardiograms were performed, and serum levels of CTRP5, procollagen type Ⅰ (PC Ⅰ), and procollagen type Ⅲ (PC Ⅲ) were measured. The patients with obstructive stenosis were divided into unstable angina (UA) group (n=67), ST-segment elevation myocardial infarction (STEMI) group (n=57), and non-ST-segment elevation myocardial infarction (NSTEMI) group (n=61) ; based on the number of coronary artery lesions, they were categorized into single-vessel disease group (n=42), double-vessel disease group (n=69), and triple-vessel disease group (n=73). According to the SYNTAXⅡ score, they were classified into low-risk (n=77), intermediate-risk (n=73), and high-risk (n=34) groups. Based on the median levels of PCⅠ, patients were divided into low PCⅠ level group (n=95) and high PC I level group (n=89) ; similarly, based on PCⅢ median levels, into low PCⅢ level group (n=93) and high PCⅢ level group (n=91). Multiuariate Logistic regression analysis was used to explore the influencing factors of coronary artery lesion severity. Receiver operating characteristic (ROC) curves were drawn to analyze the diagnostic value of serum CTRP5 for coronary artery lesion severity. Results In ACS patients, white blood cell count, neutrophil count, neutrophil-to-lymphocyte ratio, fasting blood glucose (FBG), high-sensitivity cardiac troponin Ⅰ, serum CTRP5, hypersensitive C-reactive protein (hs-CRP), PC Ⅰ, and PC Ⅲ levels were higher than in control patients (P

IntroductionMyocardial calcifications (MC) represent a relatively rare pathological process, which may accompany different cardiovascular conditions and can be broadly categorized as dystrophic or metastatic. Myocardial infarction (MI) has been traditionally regarded as the main cause of MC overall; however, no updated comprehensive data on the relative incidence of different forms of MC is available. The purpose of this systematic review of the literature is to analyze the currently available evidence on MC in terms of pathophysiology, diagnosis, and clinical presentation.Methods and resultsA total of 241 studies including a total of 368 patients affected by extensive MC were included in the final review. The majority of patients (69.8%) presented with dystrophic MC. Endomyocardial fibrosis (EMF) represents the single most common etiology of MC (24.2%), while sepsis/acute systemic inflammatory syndrome (SIRS) and chronic kidney disease were identified as the second and third most common causes respectively. The relative incidence of etiologies also varies across the years, with MI being more represented before 1990, and sepsis/SIRS becoming the single most common cause of MC after 1990. Multimodality imaging was used in the work-up of MC in 42.7% of cases. The most commonly employed imaging modality overall was echocardiography (51.9%), while after 1990 computed tomography scan became the most widely used tool (70.1%).ConclusionThe present systematic review provides new insights into the pathophysiology of MC. Previously thought to be mainly a consequence of ischemic heart disease, our data indicate that other diseases, namely EMF and sepsis/SIRS, are indeed the main conditions associated with MC. The importance of multimodality imaging in the work-up of MC is also highlighted.

Myocarditis is a polymorphic and potentially life-threatening disease characterized by a large variability in clinical presentation and prognosis. Within the broad spectrum of etiology, eosinophilic myocarditis represents a rare condition characterized by eosinophilic infiltration of the myocardium, usually associated with peripheral eosinophilia. Albeit uncommon, eosinophilic myocarditis could be potentially life-threatening, ranging from mild asymptomatic disease to multifocal widespread infiltrates associated with myocardial necrosis, thrombotic complications, and endomyocardial fibrosis. Moreover, it could progress to dilated cardiomyopathy, resulting in a poor prognosis. The leading causes of eosinophilic myocarditis are hypersensitivity reactions, eosinophilic granulomatosis with polyangiitis, cancer, hyper-eosinophilic syndrome variants, and infections. A thorough evaluation and accurate diagnosis are crucial to identifying the underlying cause and defining the appropriate therapeutic strategy. On these bases, this comprehensive review aims to summarize the current knowledge on eosinophilic myocarditis, providing a schematic and practical approach to diagnosing, evaluating, and treating eosinophilic myocarditis. [ABSTRACT FROM AUTHOR]

Purpose: This study aimed at establishing a predictive method that consists of clinical, electrocardiographic (ECG), and laboratory parameters for myocardial fibrosis, especially as detected on cardiac magnetic resonance imaging (CMRI), in patients examined with suspicion of myocarditis. Materials and Methods: This study is a retrospective, single-centre study that includes patients admitted to our centre with suspected myocarditis between March 2020 and November 2023. Participants were categorised into two groups (myocardial fibrosis positive and myocardial fibrosis negative), and a detailed comparison of comorbidities, ECG changes, and laboratory parameters was performed. Multivariate analysis was conducted to identify independent predictors of myocardial fibrosis. A nomogram was constructed using the coefficients from the multivariate analysis to estimate the probability of myocardial fibrosis presence based on key predictors. Results: This study included 98 participants with a median age of 30 years, predominantly male (80.6%), with 14.3% having hypertension, 8.2% having diabetes mellitus, and 10.2% being smokers. The myocardial fibrosis-negative group exhibited higher levels of left ventricular ejection fraction and lymphocyte count. Conversely, the myocardial fibrosis-positive group showed higher levels of ECG changes at admission, peak C-reactive protein (CRP), CRP velocity, peak troponin, N-terminal pro-brain natriuretic peptide (NT-proBNP), monocytes, and platelets (PLT). In multivariate analysis, PLT, lymphocyte, monocyte, peak troponin, and ECG changes were identified as independent predictors of myocardial fibrosis. Receiving operating characteristic (ROC) curve analysis showed the model's diagnostic accuracy for predicting myocardial fibrosis (area under the ROC (AUC): 0.959, 95% confidence interval (CI), and p<0.001). Conclusion: This comprehensive analysis highlights the relationships between clinical and laboratory parameters and myocardial fibrosis and presents a predictive model with high diagnostic accuracy. [ABSTRACT FROM AUTHOR]

Background Cardiac complications occur in 1–6% of cases of Behçet disease (BD) with intracardiac thrombus being the most frequent complication. Endomyocardial fibrosis, less common and occasionally associated with intracardiac thrombus, is reported in <20 case reports of BD, among which, three cases are described to mimic Ebstein disease based on echocardiography. We present the first case in the literature of a 34-year-old man with BD diagnosed with multiple cardiovascular complications, highlighting the challenging diagnosis and treatment of this pathology, especially regarding anticoagulation therapy. Case summary A 34-year-old man, diagnosed with BD, presented to the Emergency Room with haemoptysis. Computed tomography study of the thorax diagnosed pulmonary arterial aneurysm with multiple arterial thrombi, associated with multiple intracardiac thrombi in the right ventricle and atrium. The echocardiography confirmed the presence of voluminous thrombi in the right ventricle and atrium and showed hypertrabeculation of the right ventricle and a high insertion of the posterior leaflet of the tricuspid valve inducing a moderate tricuspid insufficiency compatible with an Ebstein disease. The cardiac MRI later revealed right ventricular fibrosis consistent with endomyocardial fibrosis and sequelae of myocarditis, also described as BD rare cardiac manifestations. The patient had a favourable outcome under anticoagulant treatment and immunosuppressive drugs. Discussion The association of multiple cardiovascular complications can occur in a single patient with BD. The endomyocardial fibrosis in the right heart chambers acting as a substrate for thrombus formation and subsequent pulmonary embolism; fibrosis extending to the tricuspid valve inducing an Ebstein-like morphology. [ABSTRACT FROM AUTHOR]

Endomyocardial fibrosis secondary to hyper-eosinophilic syndrome also known as Loeffler’s Endocarditis is a rare cause of restrictive cardiomyopathy. If left untreated, it carries a very high morbidity and mortality rate. The case of a 20 years old girl, a known case of polyarticular juvenile idiopathic arthritis since the age of 13 years was reported at Federal Government Polyclinic Hospital, Islamabad on 14th May 2022. She presented with an acute history of shortness of breath and cough for two weeks. Her initial echocardiogram showed suspicion of Loeffler’s Endocarditis, which is attributed to be an adverse effect of etanercept- a tumour necrosis factor (TNF) inhibitor, which she had been prescribed for her arthritis. The patient is currently being managed with high doses of steroids, therapeutic anticoagulation with rivaroxaban, carvedilol for tachycardia and mycophenolate mofetil as an immunosuppressant. Key Words: Endomyocardial fibrosis, Juvenile Idiopathic Arthritis (JIA), TNF alpha inhibitors, Hyper-eosinophilic syndrome (HES).

Abstract Background Endomyocardial fibrosis is a grim disease. It is the most common restrictive cardiomyopathy worldwide, but the exact etiology and pathogenesis both remain unknown. Endomyocardial fibrosis is recurrently associated with chronic eosinophilia and probable dietary, environmental, and infectious factors, which contribute not only to the onset of the disease (an inflammatory process) but also to its progression and maintenance (endomyocardial damage and scar formation). The trademark of the disease is the fibrotic obliteration of the affected ventricle. The combination of such processes produces focal or diffuse endocardial thickening and fibrosis, which leads to restrictive physiology. Endomyocardial fibrosis affects the apices of the right and the left ventricle in around 50% of cases and most often extends to the posterior leaflet of the mitral valve. Sometimes it involves the papillary muscle and chordae tendineae, causing atrioventricular valve dysfunction. The fibrosis does not affect extracardiac organs. This cardiomyopathy is most recurrent in tropical areas of the world. Case presentation A 67-year-old Black male with past medical history of schistosomiasis infection in childhood presented with progressive dyspnea, lower extremity edema, and weakness for 2 years. He was diagnosed with endomyocardial fibrosis. The echocardiogram showed an increased thickness in the septum (17 mm) and free left ventricular wall (15 mm), obliteration of the left ventricular apex and inflow tract, and mitral valve regurgitation. Cardiac magnetic resonance imaging revealed apical left ventricle wall thickening with left ventricular apical obliteration associated with enlargement of the respective atrium. Delayed enhancement imaging showed endomyocardium enhancement involving left ventricular apex, mitral valve regurgitation due to annulus dilation, and a thrombus at left ventricular apex. He underwent open heart surgery with mitral valve replacement, endocardial decortication, endomyocardiectomy, and two-vessel coronary artery bypass grafting as preoperative coronary angiogram showed mild right coronary artery and proximal left anterior descending artery severe lesions. Postoperative course was uncomplicated, and he was discharged successfully from the hospital. Six months after surgery, he was New York Heart Association functional class I. Conclusion The purpose of this case report is to illustrate the aspects of endomyocardial fibrosis by reporting a case of this entity. In conclusion, progress in imaging techniques and treatment in a reference institution for cardiac diseases contribute to earlier diagnosis and survival in patients with endomyocardial fibrosis.

Abstract Background Breast radiotherapy (RT) induces diffuse myocardial changes, which may increase the incidence of heart failure with preserved ejection fraction. This study aimed to evaluate the early signs of diffuse fibrosis after RT and their evolution during a six-year follow-up. Methods Thirty patients with early-stage left-sided breast cancer were studied with echocardiography and electrocardiography (ECG) at baseline, after RT, and at three-year and six-year follow-up visits. Echocardiography analysis included an off-line analysis of integrated backscatter (IBS). ECG was analysed for fragmented QRS (fQRS). In addition, cardiac magnetic resonance (CMR) imaging was performed at the six-year control. The left ventricle 16-segment model was used in cardiac imaging, and respective local radiation doses were analysed. Results Regional myocardial reflectivity in inferoseptal segments increased by 2.02 (4.53) dB (p = 0.026) and the percentage of leads with fQRS increased from 9.2 to 16.4% (p = 0.002) during the follow-up. In CMR imaging, abnormal extracellular volume (ECV) and T1 mapping values were found with anteroseptal and apical localization in a median of 3.5 (1.00–5.75) and 3 (1.25–4.00) segments, respectively. A higher left ventricle radiation dose was associated with an increased likelihood of having changes simultaneously in CMR and echocardiography (OR 1.26, 95% Cl. 1.00–1.59, p = 0.047). Conclusions After radiotherapy, progressive changes in markers of diffuse myocardial fibrosis were observed in a multimodal manner in ECG and echocardiography. Changes in echocardiography and abnormal values in CMR were localized in the septal and apical regions, and multiple changes were associated with higher radiation doses.

Background: Endomyocardial fibrosis is a grim disease. It is the most common restrictive cardiomyopathy worldwide, but the exact etiology and pathogenesis both remain unknown. Endomyocardial fibrosis is recurrently associated with chronic eosinophilia and probable dietary, environmental, and infectious factors, which contribute not only to the onset of the disease (an inflammatory process) but also to its progression and maintenance (endomyocardial damage and scar formation). The trademark of the disease is the fibrotic obliteration of the affected ventricle. The combination of such processes produces focal or diffuse endocardial thickening and fibrosis, which leads to restrictive physiology. Endomyocardial fibrosis affects the apices of the right and the left ventricle in around 50% of cases and most often extends to the posterior leaflet of the mitral valve. Sometimes it involves the papillary muscle and chordae tendineae, causing atrioventricular valve dysfunction. The fibrosis does not affect extracardiac organs. This cardiomyopathy is most recurrent in tropical areas of the world. Case presentation: A 67-year-old Black male with past medical history of schistosomiasis infection in childhood presented with progressive dyspnea, lower extremity edema, and weakness for 2 years. He was diagnosed with endomyocardial fibrosis. The echocardiogram showed an increased thickness in the septum (17 mm) and free left ventricular wall (15 mm), obliteration of the left ventricular apex and inflow tract, and mitral valve regurgitation. Cardiac magnetic resonance imaging revealed apical left ventricle wall thickening with left ventricular apical obliteration associated with enlargement of the respective atrium. Delayed enhancement imaging showed endomyocardium enhancement involving left ventricular apex, mitral valve regurgitation due to annulus dilation, and a thrombus at left ventricular apex. He underwent open heart surgery with mitral valve replacement, endocardial decortication, endomyocardiectomy, and two-vessel coronary artery bypass grafting as preoperative coronary angiogram showed mild right coronary artery and proximal left anterior descending artery severe lesions. Postoperative course was uncomplicated, and he was discharged successfully from the hospital. Six months after surgery, he was New York Heart Association functional class I. Conclusion: The purpose of this case report is to illustrate the aspects of endomyocardial fibrosis by reporting a case of this entity. In conclusion, progress in imaging techniques and treatment in a reference institution for cardiac diseases contribute to earlier diagnosis and survival in patients with endomyocardial fibrosis. [ABSTRACT FROM AUTHOR]

Background: Breast radiotherapy (RT) induces diffuse myocardial changes, which may increase the incidence of heart failure with preserved ejection fraction. This study aimed to evaluate the early signs of diffuse fibrosis after RT and their evolution during a six-year follow-up. Methods: Thirty patients with early-stage left-sided breast cancer were studied with echocardiography and electrocardiography (ECG) at baseline, after RT, and at three-year and six-year follow-up visits. Echocardiography analysis included an off-line analysis of integrated backscatter (IBS). ECG was analysed for fragmented QRS (fQRS). In addition, cardiac magnetic resonance (CMR) imaging was performed at the six-year control. The left ventricle 16-segment model was used in cardiac imaging, and respective local radiation doses were analysed. Results: Regional myocardial reflectivity in inferoseptal segments increased by 2.02 (4.53) dB (p = 0.026) and the percentage of leads with fQRS increased from 9.2 to 16.4% (p = 0.002) during the follow-up. In CMR imaging, abnormal extracellular volume (ECV) and T1 mapping values were found with anteroseptal and apical localization in a median of 3.5 (1.00–5.75) and 3 (1.25–4.00) segments, respectively. A higher left ventricle radiation dose was associated with an increased likelihood of having changes simultaneously in CMR and echocardiography (OR 1.26, 95% Cl. 1.00–1.59, p = 0.047). Conclusions: After radiotherapy, progressive changes in markers of diffuse myocardial fibrosis were observed in a multimodal manner in ECG and echocardiography. Changes in echocardiography and abnormal values in CMR were localized in the septal and apical regions, and multiple changes were associated with higher radiation doses. [ABSTRACT FROM AUTHOR]

Endomyocardial fibrosis (EMF) affects approximately 12 million persons worldwide and is an important cause of restrictive cardiomyopathy in the developing world, with the highest prevalence reported in sub-Saharan Africa, South Asia, and South America. EMF is characterized by apical infiltration with fibrotic tissue in one or both ventricles, often associated with thrombus in early stage of the disease, calcification in late stage of the disease, and typical symptoms of restrictive heart failure. Clinical evaluation, transthoracic echocardiography, and characteristic Doppler findings of restrictive physiology are sufficient to diagnose EMF in most of the cases. However, few cases may require cardiac magnetic resonance due to poor echo window or shadowing due to apical calcification. Tissue characterization ability of cardiac magnetic resonance imaging has evolved further in the era of parametric mapping. We plan to showcase the incremental value of parametric mapping (T1/T2 mapping) in staging, prognostication, and management in two cases of EMF.

Myocarditis is a polymorphic and potentially life-threatening disease characterized by a large variability in clinical presentation and prognosis. Within the broad spectrum of etiology, eosinophilic myocarditis represents a rare condition characterized by eosinophilic infiltration of the myocardium, usually associated with peripheral eosinophilia. Albeit uncommon, eosinophilic myocarditis could be potentially life-threatening, ranging from mild asymptomatic disease to multifocal widespread infiltrates associated with myocardial necrosis, thrombotic complications, and endomyocardial fibrosis. Moreover, it could progress to dilated cardiomyopathy, resulting in a poor prognosis. The leading causes of eosinophilic myocarditis are hypersensitivity reactions, eosinophilic granulomatosis with polyangiitis, cancer, hyper-eosinophilic syndrome variants, and infections. A thorough evaluation and accurate diagnosis are crucial to identifying the underlying cause and defining the appropriate therapeutic strategy. On these bases, this comprehensive review aims to summarize the current knowledge on eosinophilic myocarditis, providing a schematic and practical approach to diagnosing, evaluating, and treating eosinophilic myocarditis.

Objective To investigate the relationship between levels of homocysteine (Hcy) and left ventricular myocardial fibrosis measured by late gadolinium enhancement (CMR-LGE) in hypertensive patients. Methods Eightyseven hypertensive patients who underwent cardiac magnetic resonance (CMR) examination were selected. According to Hcy level, patients were divided into the normal Hcy group (Hcy≤15 µmol/L, 40 cases) and the high Hcy group (Hcy＞15 µmol/L, 47 cases). The clinical data of all patients were completed between the two groups. Spearman rank correlation analysis was used to analyze the correlation between Hcy and late gadolinium enhancement volume percentage (LGE% ), and the correlation between Hcy and other cardiac parameters. Results The left atrial diameter (LAD), left ventricular enddiastolic diameter (LVEDD), left ventricular end-diastolic volume (LVEDV), left ventricular end-systolic volume (LVESV), left ventricular mass (LVM), left ventricular mass index (LVMI) and LGE% were significantly higher in the high Hcy group than those in the normal Hcy group. The left ventricular ejection fraction (LVEF) was significantly lower in the high Hcy group than that of the normal Hcy group (P＜0.05). Spearman correlation analysis showed that Hcy was positively correlated with LAD, LVEDD, LVEDV, LVESV, LVM, LVMI and LGE% (rs were 0.356, 0.332, 0.288, 0.383, 0.338, 0.325, 0.391, respectively, all P＜0.01). Hcy was negatively correlated with LVEF (rs=-0.412, P＜0.01). Conclusion Hypertensive patients with high Hcy level have severe left ventricular myocardial fibrosis, resulting in more serious left heart structure and dysfunction. [ABSTRACT FROM AUTHOR]

ABSTRACT Endomyocardial fibrosis is a neglected tropical disease that leads to restrictive cardiomyopathy. Its etiopathogenis is unclear and involves the progression of 3 stages of the disease. Compared with echocardiography, cardiac magnetic resonance imaging shows better apical visualization of obliteration and thrombus and provides an early diagnosis. However, there is no specific drug therapy, although surgery can increase survival. Therefore, surgical resection of the fibrous and thickened endocardium is recommended for symptomatic patients. The risk of mortality increases as the ratio of endocardial fibrous tissue per body surface rises. The aim of this manuscript is to describe the surgical management of the right-sided endomyocardial fibrosis mimicking tumor with recurrent pulmonary embolism.

Objective To investigate the effect of the conserved long non-coding RNA myocardial infarction associate transcript (lncRNA MIAT) targeting and regulating miR-128-3p on ventricular remodeling and myocardial fibrosis in atrial fibrillation (AF) in rats. Methods The calcium chloride-acetylcholine mixture was injected into the sublingual vein to induce AF rat models. The rat models were divided into five groups by random number table method: the model group, the lncRNA MIAT siRNA plasmid group (MIAT group), the miR-128-3p siRNA plasmid group (miR-128-3p group), the lncRNA MIAT siRNA plasmid + miR-128-3p siRNA plasmid group (MIAT+miR-128-3p group) and the empty plasmid group, with 12 rats in each group. Another 12 rats were given with the same dose of normal saline through sublingual vein and were used as the control group. After intervention, the atrial myocardial electrophysiological level of rats was detected, and the effective refractory period (ERP) and 90% action potential duration (APD90) were compared between groups. The left ventricular mass index was also measured in five groups of rats. Sirius red staining was used to detect the degree of myocardial tissue fibrosis in rats. The myocardial collagen volume fraction (CVF) was compared between groups. Serum levels of interleukin (IL) -18, IL-6 and transforming growth factor- β1 (TGF- β1) were detected by kit. Real-time quantitative PCR (qRT-PCR) was used to detect the expression of miR-128-3p in myocardial tissues in each group. Dual luciferase reporter gene assay was used to detect the targeted regulation effect of lncRNA MIAT on miR-128-3p. Results Compared with the control group, the ERP, APD90, and myocardial tissue miR-128-3p expression were significantly reduced in the model group (P＜0.05), and the left ventricular mass index, CVF, serum IL-18, IL-6 and TGF-β1 levels were significantly increased (P＜0.05). Compared with the model group and the lncRNA MIAT siRNA plasmid+miR-128- 3p siRNA plasmid group, the ERP, APD90, and myocardial tissue miR-128-3p expression were significantly increased in the lncRNA MIAT siRNA plasmid group (P＜0.05), and the left ventricular mass index, CVF, serum IL-18, IL-6 and TGF)β1 levels were significantly reduced (P＜0.05). The ERP, APD90 and myocardial tissue miR-128-3p expression were significantly decreased in the miR-128-3p siRNA plasmid group (P＜0.05), and the left ventricular mass index, CVF, serum IL-18, IL-6 and TGF- β1 levels were significantly increased (P＜0.05). There were no significant differences in the indicators between the empty plasmid groups (P＞0.05). LncRNA MIAT could down-regulate the expression of miR-128-3p by targeting. Conclusion LncRNA MIAT can participate in the pathogenesis of AF by targeting down-regulation of miR)128-3p expression. The down-regulation of lncRNA MIAT can inhibit inflammation by promoting miR-128-3p expression, reduce myocardial fibrosis in AF rats, improve the atrial myocardial electrophysiological level and delay the process of ventricular remodeling in rats. [ABSTRACT FROM AUTHOR]

Background Endomyocardial fibrosis (EMF) is a rare and underdiagnosed cause of restrictive cardiomyopathy. Its aetiology is not yet defined and could be caused by the influence of different clinical factors that seem to combine with genetic aspects of individuals susceptible to an inflammatory process that leads to formation of fibrosis. Case summary We describe a case of a 50-year-old man from the northeastern region of Brazil, where there is high prevalence of schistosomiasis. He presented to our centre with symptoms of right heart failure. The echocardiogram showed normal left ventricular ejection fraction. Right ventricular had normal systolic function but in the apical region was filled with a homogeneous and hypoechoic image causing obliteration and restriction of the apex. The late gadolinium enhancement with cardiac magnetic resonance showed diffuse and heterogeneous subendocardial fibrosis in the right ventricle apex consistent with EMF, but declined endocardiectomy. Discussion This report presents an interesting case of EMF and schistosomiasis simultaneously. The hypothesis of parasitosis as a probable cause of EMF was raised by helminth-induced hypereosinophilia. Complementary imaging tests such as magnetic resonance imaging and echocardiography, in addition to clinical and epidemiological suspicion, are essential for its diagnosis. Early surgical resolution becomes crucial for long-term survival. [ABSTRACT FROM AUTHOR]

Background: Hypereosinophilic syndrome can produce cardiac involvement and endomyocardial fibrosis, which have a poor prognosis. However, there is limited information regarding cardiac involvement among migrants from Latin America with eosinophilia related to helminthiasis., Methods: We conducted a pilot observational study where an echocardiography was performed on migrants from Latin America with both eosinophilia (>450 cells/μL) and a diagnosis of helminth infection, and on migrants from Latin America without eosinophilia or helminth infection. Microbiological techniques included a stool microscopic examination using the Ritchie's formalin-ether technique, and a specific serology to detect Strongyloides stercoralis antibodies., Results: 37 participants were included, 20 with eosinophilia and 17 without eosinophilia. Twenty (54.1%) were men with a mean age of 41.3 (SD 14.3) years. Helminthic infections diagnosed in the group with eosinophilia were: 17 cases of S. stercoralis infection, 1 case of hookworm infection, and 2 cases of S. stercoralis and hookworm coinfection. Among participants with eosinophilia, echocardiographic findings revealed a greater right ventricle thickness (p = 0.001) and left atrial area and volume index (p = 0.003 and p = 0.004, respectively), while showing a lower left atrial strain (p = 0.006) and E-wave deceleration time (p = 0.008). An increase was shown in both posterior and anterior mitral leaflet thickness (p = 0.0014 and p = 0.004, respectively) when compared with participants without eosinophilia., Conclusions: Migrants from Latin America with eosinophilia related to helminthic infections might present incipient echocardiographic alterations suggestive of early diastolic dysfunction, that could be related to eosinophilia-induced changes in the endomyocardium., Competing Interests: The authors have declared that no competing interests exist., (Copyright: © 2024 Sempere et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Introduction: Myocardial calcifications (MC) represent a relatively rare pathological process, which may accompany different cardiovascular conditions and can be broadly categorized as dystrophic or metastatic. Myocardial infarction (MI) has been traditionally regarded as the main cause of MC overall; however, no updated comprehensive data on the relative incidence of different forms of MC is available. The purpose of this systematic review of the literature is to analyze the currently available evidence on MC in terms of pathophysiology, diagnosis, and clinical presentation., Methods and Results: A total of 241 studies including a total of 368 patients affected by extensive MC were included in the final review. The majority of patients (69.8%) presented with dystrophic MC. Endomyocardial fibrosis (EMF) represents the single most common etiology of MC (24.2%), while sepsis/acute systemic inflammatory syndrome (SIRS) and chronic kidney disease were identified as the second and third most common causes respectively. The relative incidence of etiologies also varies across the years, with MI being more represented before 1990, and sepsis/SIRS becoming the single most common cause of MC after 1990. Multimodality imaging was used in the work-up of MC in 42.7% of cases. The most commonly employed imaging modality overall was echocardiography (51.9%), while after 1990 computed tomography scan became the most widely used tool (70.1%)., Conclusion: The present systematic review provides new insights into the pathophysiology of MC. Previously thought to be mainly a consequence of ischemic heart disease, our data indicate that other diseases, namely EMF and sepsis/SIRS, are indeed the main conditions associated with MC. The importance of multimodality imaging in the work-up of MC is also highlighted., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2024 Sozzi, Gnan, Faggiano, Giangiacomi, Iacuzio, Canetta, Santangelo, Pisaniello, Eker and Carugo.)

Endomyocardial fibrosis (EMF) is characterized by fibrous tissue deposition on the endocardial surface leading to impaired filling of one or both ventricles, resulting in either right or left heart failure or both. Although Sinus node dysfunction and tachyarrhythmia - atrial fibrillation, ventricular tachycardia, have been commonly reported, complete heart block (CHB) necessitating a pacemaker is rare in EMF. Transvenous pacing is technically limited by fibrotic obliteration of the affected ventricle that results in poor lead parameters, and alternative pacing strategy like epicardial pacing may be required in many. We report three cases of EMF, who were treated with an alternative pacing strategy.

The long-term results of transatrial transpulmonary tetralogy of Fallot (TOF) repair have been excellent. Progressive pulmonary regurgitation and consequent right ventricular (RV) dilatation are the most common long-term sequel of definitive repair in childhood. Overt systemic venous congestion after TOF repair is limited to the rare setting where RV dysfunction sets in due to deferred surgery or progressive arrhythmia. Here, we report a unique case of right heart failure from an unexpected etiology, 28 years after TOF repair. Cardiac catheterization confirmed findings of elevated right heart pressures. Magnetic resonance imaging showed obliteration of the RV apex with late gadolinium enhancement of the right ventricular apical endomyocardium.

Endomyocardial fibrosis (EMF) is a disease known to cause restrictive cardiomyopathy. It shows a high prevalence in tropical countries. Several triggering factors have been proposed. However, the pathogenesis is still a mystery. The disease is progressive, and the outcome is generally unfavorable. The most common symptom is heart failure. However, an atypical presentation may be expected. Our case presented with symptoms suggestive of ischemia and missed diagnosis initially as ischemic cardiomyopathy. This report aims to increase the attention and awareness of this disease. We present a case of a 53‐year‐old man referred to the emergency department for sudden chest pain, left‐sided and non‐radiating lasted for several minutes, awoke him from sleep with no associated symptoms. He is known to have Diabetes type‐2 and hypertension on oral therapy. Cardiac markers were within the normal limit. The patient was discharged home with an appointment at the cardiology outpatient clinic. Echocardiography was done and revealed mildly reduced left ventricular (LV) systolic function, Ejection Fraction of 46%, asymmetric LV hypertrophy affecting the apical segments with aneurysm, and calcified apical thrombus. CT coronary angiography was done with non‐significant Left Anterior Descending artery lesions and left ventricular hypertrophy affecting the apex with calcified apical thrombus. Further investigation by cardiac MRI revealed apical thrombus and late apical uptake suggesting Endomyocardial Fibrosis of possible eosinophilic etiology. The patient continued to have attacks of similar chest pain, for which stress cardiac MRI was done and was negative for ischemia. Another diagnostic workup was done, including hematological and serological tests such as Antinuclear Antibodies and Schistosoma Antibodies. The patient was kept on valsartan and Bisoprolol with oral anticoagulant (vitamin K antagonist) and Rosuvastatin. EMF may have a heterogeneous presentation and should be considered in a patient with calcific apical thrombus without previous history of cardiac problem, even in the non‐tropical region. [ABSTRACT FROM AUTHOR]

Infiltrative cardiomyopathies are abnormal accumulations or depositions of different substances in cardiac tissue leading to its dysfunction, first diastolic, then systolic. The different infiltrative cardiomyopathies are amyloidosis (both light chain amyloidosis and transthyretin amyloidosis variants), lysosomal and glycogen storage disorders (Fabry-Anderson disease), and iron overload (hemochromatosis and thalassemia associated with blood transfusions), as well as inflammatory diseases such as sarcoidosis. We also evoke hypereosinophilic syndrome associated with endomyocardial fibrosis. Echocardiography is the first essential step after interrogatory and clinical examination and may help the cardiologist as a screening tool. Cardiac MRI is the second fundamental step towards the diagnosis especially due to the late gadolinium enhancement and to the T1-mapping. Cardiac amyloidosis diagnosis also requires the use of nuclear imaging. Cardiac CT-Scan may be useful for estimating the amyloid load, identify potential cardiac thrombus and rule out associated coronaropathy. [ABSTRACT FROM AUTHOR]

Abstract Endomyocardial fibrosis (EMF) is a disease known to cause restrictive cardiomyopathy. It shows a high prevalence in tropical countries. Several triggering factors have been proposed. However, the pathogenesis is still a mystery. The disease is progressive, and the outcome is generally unfavorable. The most common symptom is heart failure. However, an atypical presentation may be expected. Our case presented with symptoms suggestive of ischemia and missed diagnosis initially as ischemic cardiomyopathy. This report aims to increase the attention and awareness of this disease. We present a case of a 53‐year‐old man referred to the emergency department for sudden chest pain, left‐sided and non‐radiating lasted for several minutes, awoke him from sleep with no associated symptoms. He is known to have Diabetes type‐2 and hypertension on oral therapy. Cardiac markers were within the normal limit. The patient was discharged home with an appointment at the cardiology outpatient clinic. Echocardiography was done and revealed mildly reduced left ventricular (LV) systolic function, Ejection Fraction of 46%, asymmetric LV hypertrophy affecting the apical segments with aneurysm, and calcified apical thrombus. CT coronary angiography was done with non‐significant Left Anterior Descending artery lesions and left ventricular hypertrophy affecting the apex with calcified apical thrombus. Further investigation by cardiac MRI revealed apical thrombus and late apical uptake suggesting Endomyocardial Fibrosis of possible eosinophilic etiology. The patient continued to have attacks of similar chest pain, for which stress cardiac MRI was done and was negative for ischemia. Another diagnostic workup was done, including hematological and serological tests such as Antinuclear Antibodies and Schistosoma Antibodies. The patient was kept on valsartan and Bisoprolol with oral anticoagulant (vitamin K antagonist) and Rosuvastatin. EMF may have a heterogeneous presentation and should be considered in a patient with calcific apical thrombus without previous history of cardiac problem, even in the non‐tropical region.

BackgroundFirst described in Uganda over seven decades ago, Endomyocardial fibrosis (EMF) is a rare form of restrictive cardiomyopathy found in the tropics. EMF occurs mainly in two phenotypes; biventricular involvement and right ventricular (RV) form. Previously endemic in several countries, there are reports suggesting that the disease is on the decline.ObjectivesTo describe trends in annual incidence rates of newly diagnosed EMF cases at the Uganda Heart Institute (UHI).MethodsThis was a retrospective chart review of all newly diagnosed EMF cases at UHI from January 2007 to December 2020. Cases were divided into two groups A (2007–2013) and B (2014–2020).ResultsA total of 155 cases were diagnosed during the period (Group A, n = 124; Group B, n = 31). There were no significant differences between the two groups A and B regarding median age at diagnosis (14 vs. 12 years, p = 0.0940), gender (48.4% female vs. 35.5%, p = 0.1987), and EMF type (66.9% RV EMF vs. 71.0%, p = 0.6634), respectively. The presence of complications such as intracardiac thrombus (5.6 vs. 32.2%, p = 0.0002) and pericardial effusion (57.3% vs. 80.6, p = 0.0172) were more frequent in group B than A, respectively. Pulmonary hypertension (PHT) was predominantly seen in cases with biventricular EMF compared to those with RV EMF (26 vs. 3.8%, p = 0.0001). The number of new cases diagnosed per year remained largely stable in the period 2007–2011, ranging 14–21 per year, peaked in 2012 (26 new cases), and thereafter declined from 10 cases seen in 2013 to 1–5 cases seen per year in the period 2017–2020. Similarly, the annual incidence rates of new EMF diagnosis remained relatively stable in the period 2007–2012, ranging between 22.7 and 29.7 per 10,000 patients seen in the echo labs, and then dramatically declined after 2012 to range between 1.0 and 4.5 new cases per 10,000 patients in the period between 2017 and 2020.ConclusionThere has been a steady decline in the number of new cases of EMF seen at the UHI. However, there were no significant differences in the gender, age at diagnosis and EMF subtype of cases during the period under review. Complication rates were more frequent in the later cohort.

Tropical endomyocardial fibrosis (EMF) is endemic in southern districts of Kerala state in India, and sporadic cases are reported from other parts of Kerala. Transthoracic echocardiogram (TTE) is the initial imaging tool and is diagnostic for this condition. The inherent limitation of TTE is its limited sensitivity in the detection of intracardiac thrombus. Transthoracic echocardiography, contrast-enhanced cardiac computed tomography, and cardiac magnetic resonance imaging (CMR) are the imaging modalities for the detection of intracardiac thrombus. This report describes a 46-year-old male with right ventricular EMF (RVEMF) who presented with right heart failure. The TTE diagnosed RVEMF but failed to demonstrate the right atrial thrombus which was clearly seen on CMR. The merits of different imaging modalities for the detection of intracardiac thrombus are discussed.

Restrictive cardiomyopathy (RCM) includes a heterogeneous group of diseases that cause increased myocardial stiffness, leading to impaired ventricular relaxation and severe diastolic dysfunction. Given that it is the least common type of cardiomyopathy, it can be a diagnostic challenge due to its varied pathogenesis, clinical presentation, and diagnostic evaluation. In this review, we provide an overview of different etiologies of RCM and examine the diagnostic and treatment approaches for various types. [ABSTRACT FROM AUTHOR]

Introduction: Cardiac masses have a wide range of etiologies with the most common being thrombi and less commonly tumors. However, in Sub-Saharan Africa other etiologies not commonly seen in developed countries such as endomyocardial fibrosis (EMF) must be considered. EMF is a disease process associated with poverty, a poor diet, and eosinophilia although its pathology is poorly understood. Case report: We report a case of a 53-year-old male with a history of dilated cardiomyopathy who presented to a Ugandan Emergency Department in respiratory distress. Bedside echocardiography was performed which revealed a large mass in the apex of the left ventricle. The patient was subsequently given supplemental oxygen and intravenous furosemide, however he later died while in the emergency department due to limited resources and lack of definitive care. Discussion: The list of potential etiologies of cardiac masses is widely variable, and in settings such as Sub-Saharan Africa, this list must be expanded to include possible diagnoses such as EMF. EMF is a diagnosis that should be considered in patients presenting with respiratory distress and a cardiac mass present on echocardiography, such as the case presented here. The limited opportunities for medical personnel to diagnose cardiovascular disease can be made more efficient by the use of diagnostic imaging devices which are portable, yet capable of diagnosing the most common local pathologies [9-11].

We present a case of late presentation nontropical endomyocardial fibrosis isolated to the right ventricle and tricuspid valve (TV). In response to deteriorating hemodynamics, surgical debulking and TV removal were performed before initiation of centralized venoarterial extracorporeal membrane oxygenation support. Definitive endomyocardial resection with a TV prosthesis was then successfully completed. (Level of Difficulty: Advanced.)

Background: Cardiac fibrosis is a lethal outcome of excessive formation of myofibroblasts that are scar-forming cells accumulated after heart injury. It has been reported that cardiac endothelial cells (ECs) contribute to a substantial portion of myofibroblasts through endothelial to mesenchymal transition (EndoMT). Recent lineage tracing studies demonstrate that myofibroblasts are derived from the expansion of resident fibroblasts rather than from the transdifferentiation of ECs. However, it remains unknown whether ECs can transdifferentiate into myofibroblasts reversibly or EndoMT genes were just transiently activated in ECs during cardiac fibrosis.Methods: By using the dual recombination technology based on Cre-loxP and Dre-rox, we generated a genetic lineage tracing system for tracking EndoMT in cardiac ECs. We used it to examine if there is transiently activated mesenchymal gene expression in ECs during cardiac fibrosis. Activation of the broadly used marker gene in myofibroblasts, αSMA (α-smooth muscle actin), and the transcription factor that induces epithelial to mesenchymal transition, Zeb1 (zinc finger E-box-binding homeobox 1), was examined.Results: The genetic system enables continuous tracing of transcriptional activity of targeted genes in vivo. Our genetic fate mapping results revealed that a subset of cardiac ECs transiently expressed αSMA and Zeb1 during embryonic valve formation and transdifferentiated into mesenchymal cells through EndoMT. Nonetheless, they did not contribute to myofibroblasts, nor transiently expressed αSMA or Zeb1 after heart injury. Instead, expression of αSMA was activated in resident fibroblasts during cardiac fibrosis.Conclusions: Mesenchymal gene expression is activated in cardiac ECs through EndoMT in the developing heart, but ECs do not transdifferentiate into myofibroblasts, nor transiently express some known mesenchymal genes during homeostasis and fibrosis in the adult heart. Resident fibroblasts that are converted to myofibroblasts by activating mesenchymal gene expression are the major contributors to cardiac fibrosis. [ABSTRACT FROM AUTHOR]

Objective: Rare disease Background: The cardiotoxic effects of chemotherapy in cancer treatment can damage cardiomyocytes. A common link in the pathogenesis is the proliferation of fibroblasts and the increase of collagen synthesis, leading to development of common endomyocardial fibrosis. The walls of ventricles become rigid and their inability to relax prevents them from carrying the required amount of blood. The myocardial contractility gradually decreases and leads to ventricular dysfunction and signs of heart failure. Case Report: A 29-year-old woman with reduced exercise tolerance, dyspnea, and heart rhythm disorders was admitted to our hospital. Lymphoblastic leukemia had been diagnosed at the age of 8 years, and she underwent 8 courses of polychemotherapy. She had normal heart anatomy. At the current admission, the diagnostic protocol included echocardiography, computed tomography, cardiac catheterization, and angiocardiography. She was diagnosed with restrictive cardiomyopathy with isolated endomyocardial fibrosis of the right ventricle, and moderate tricuspid valve insufficiency NYHA class III. The patient underwent a right-sided bidirectional cavopulmonary connection with tricuspid valve repair. The early postoperative period was uneventful, and SVCp decreased to 14 mmHg. At discharge, the patient's clinical condition had improved and tricuspid regurgitation was minimal. Conclusions: The one-and-a-half ventricular correction, commonly used in patients with Ebstein's anomaly and RV dysfunction or in patients with congenital heart defects associated with RV hypoplasia, is proposed as the method of choice for cardiomyopathy type RV dysfunction. [ABSTRACT FROM AUTHOR]

Myocardial fibrosis (MF) is the result of persistent and repeated aggravation of myocardial ischemia and hypoxia, leading to the gradual development of heart failure of chronic ischemic heart disease. Triptolide (TPL) is identified to be involved in the treatment for MF. This study aims to explore the mechanism of TPL in the treatment of MF. The MF rat model was established, subcutaneously injected with isoproterenol and treated by subcutaneous injection of TPL. The cardiac function of each group was evaluated, including LVEF, LVFS, LVES, and LVED. The expressions of ANP, BNP, inflammatory related factors (IL-1β, IL-18, TNF-α, MCP-1, VCAM- 1), NLRP3 inflammasome factors (NLRP3, ASC) and fibrosis related factors (TGF-β1, COL1, and COL3) in rats were dete cted. H&E staining and Masson staining were used to observe myocardial cell inflammation and fibrosis of rats. Western blot was used to detect the p-P65 and t-P65 levels in nucleoprotein of rat myocardial tissues. LVED and LVES of MF group were significantly upregulated, LVEF and LVFS were significantly downregulated, while TPL treatment reversed these trends; TPL treatment downregulated the tissue injury and improved the pathological damage of MF rats. TPL treatment downregulated the levels of inflammatory factors and fibrosis factors, and inhibited the activation of NLRP3 inflammasome. Activation of NLRP3 inflammasome or NF-κB pathway reversed the effect of TPL on MF. Collectively, TPL inhibited the activation of NLRP3 inflammasome by inhibiting NF-αB pathway, and improved MF in MF rats. [ABSTRACT FROM AUTHOR]

Pericardial constriction is a condition, in which there is a lack of transmission of changes in intrathoracic pressures to the structures encased in pericardium due to thickened, fibrosed, and most of the cases, calcified pericardium. A 24-year-old male presented to us in right heart failure. There was a history of diagnosis of acute rheumatic fever with carditis in childhood. X-ray and echocardiogram gave us a probable diagnosis of rheumatic constrictive pericarditis (CP) with mitral regurgitation (MR). The diagnosis of CP was confirmed with computed tomography chest and invasive hemodynamic assessment. He had significantly elevated pulmonary artery (PA) pressures and elevated left ventricular dip diastolic pressures, which were unexpected findings. We have discussed the probable causes of associated MR and elevated PA pressures in this case report. We have also tried to differentiate CP from restrictive cardiomyopathy, specifically endomyocardial fibrosis, which is common in the state of Kerala.

Abstract Background Endomyocardial fibrosis (EMF) represents the most common cause of restrictive cardiomyopathy worldwide. Despite a high prevalence in tropical regions, it occasionally occurs in patients who have never visited these areas. While researches have proposed various possible triggers for EMF, etiology and pathogenesis remain largely unknown. Diagnosis is based on patient history, heart failure symptoms, and echocardiographic signs of restrictive ventricular filling, atrioventricular valve regurgitation and frequently apical thrombus. Following is a case report of an Austrian patient with EMF who eventually had to undergo a heart transplant. This case report strives to promote awareness for this in non-tropical areas uncommon but nevertheless detrimental disease. Case presentation A 40-year-old woman was presented at our emergency department with chest pain and fever up to 38.1° Celsius. Plasma troponin-T levels and inflammatory markers were slightly elevated, but the echocardiogram was without pathological findings. The patient was hospitalized on the suspicion of acute myocarditis and discharged soon after improvement. Eight months later, she was presented again with chest pain and symptoms of heart failure. The echocardiogram showed normal systolic left ventricular (LV) function with LV wall thickening and severe restrictive mitral regurgitation as well as aortic and tricuspid regurgitation. Coronary angiogram was normal but right heart catheterization showed pulmonary hypertension due to left heart disease. Further diagnostic workup with cardiac magnetic resonance imaging revealed subendocardial late enhancement and apical thrombus formation in the left ventricle compatible with the diagnosis of EMF. A comprehensive diagnostic workup showed no evidence of infection, systemic immunologic or hematological disease, in particular hypereosinophilic syndrome. After a multidisciplinary consideration of several therapeutic options, the patient was listed for heart transplantation. On the waiting list, she deteriorated rapidly due to progressive heart failure and finally underwent a heart transplantation. Histological examination confirmed the diagnosis of EMF. Six years after her heart transplantation, the patient was presented in an excellent clinical condition. Conclusions Even in non-tropical regions, the diagnosis of EMF should always be considered in restrictive cardiomyopathy. Knowledge of the distinct phenotype of EMF facilitates diagnosis, but comprehensive workup and therapeutic management remain challenging and require a multidisciplinary approach.

Abstract Background Endomyocardial fibrosis (EMF) is a rare condition and a major cause of death in tropical countries. The etiology of EMF remains elusive, and no specific treatment has been developed yet, therefore it carries poor prognosis. Case presentation An 81-year-old male Chinese patient with a history of long-standing exertional breathlessness, presented with worsening symptoms rapidly evolving to orthopnea. A proper specific treatment was prescribed to the patient in the following days, including diuretics, angiotensin-converting-enzyme inhibitor and beta blockers. The patient died of progressive multiple organ failure. Conclusion Echocardiography is technically limited due to the acoustic shadowing as a result of the calcification. Chest computed tomography is a more accurate diagnostic tool to examine the anatomic distribution and extent of endomyocardial calcification in this rare case.

Endomyocardial fibrosis is a neglected tropical disease that leads to restrictive cardiomyopathy. Its etiopathogenis is unclear and involves the progression of 3 stages of the disease. Compared with echocardiography, cardiac magnetic resonance imaging shows better apical visualization of obliteration and thrombus and provides an early diagnosis. However, there is no specific drug therapy, although surgery can increase survival. Therefore, surgical resection of the fibrous and thickened endocardium is recommended for symptomatic patients. The risk of mortality increases as the ratio of endocardial fibrous tissue per body surface rises. The aim of this manuscript is to describe the surgical management of the right-sided endomyocardial fibrosis mimicking tumor with recurrent pulmonary embolism [ABSTRACT FROM AUTHOR]

Background: Endomyocardial biopsy is the gold standard and has a definite role in the surveillance of cardiac allograft rejection. Its role in other cardiac diseases is limited. However, it is required for conclusive diagnosis of a few entities in which it can influence patient management. There is no reported data regarding the utility of endomyocardial biopsy in the Indian population. Thus, this study was undertaken in a tertiary care center in India to assess the utility of endomyocardial biopsy in various cardiac diseases in the context of clinical diagnoses. Methods: All endomyocardial biopsies conducted over a 27-year period were evaluated. Clinical details including indication for biopsy were collected. Histopathological findings were recorded and classified as definitive diagnosis, probable diagnosis with features consistent with the clinical diagnosis, and nonspecific morphological findings. Results: A total of 927 endomyocardial biopsies from 719 patients were reviewed. Endomyocardial biopsy was diagnostic in 12.5% of native cardiac biopsies and 52.1% showed nondiagnostic findings. The most frequent diagnoses were amyloidosis (58.7%) and myocarditis (8.6%). Endomyocardial biopsy had a diagnostic role in evaluation of restrictive cardiac diseases. Endomyocardial fibrosis and tubercular myocarditis, relatively more prevalent in the Indian population, were also identified. Cases of rheumatic heart disease, desmin cardiomyopathy, and microfibrillar cardiomyopathy were surprise findings, proving the usefulness of endomyocardial biopsy in detecting some rare cardiac conditions. Conclusion: Endomyocardial biopsy is an important tool for the diagnosis of specific cardiac diseases including some rare entities, and for conditions which are more prevalent in our country, requiring biopsy confirmation. [ABSTRACT FROM AUTHOR]

Endomyocardial fibrosis (EMF), a restrictive cardiomyopathy characterized by subendocardial fibrosis, is commonly seen in tropical and subtropical regions. EMF involving the left ventricle presents with severe pulmonary hypertension (PH) and is a rare cause of PH in non-tropical areas. Multimodality imaging is important for accurate diagnosis, especially cardiac magnetic resonance imaging which is the cornerstone. Herein, we report the case of a patient who presented with heart failure symptoms and severe PH, and in whom EMF was diagnosed by multimodality imaging. [ABSTRACT FROM AUTHOR]

Objective: Endomyocardial fibrosis (EF) is an unusual restrictive cardiomyopathy. In Latin America there are few reports. Here, we made a description of patients diagnosed with EF in Colombia. Method: We conducted a search in the records of cardiac magnetic resonance imaging (MRI) performed in our institution between 2016-2019 looking for patients with a diagnosis of EF; sociodemographic, clinical and imaging characteristics were described. Results: Nine patients were diagnosed with EF (66.7% female), with an average age of 69 years. Patients presented an average evolution of 2.6 years. The main reported symptom was dyspnea, followed by syncope, chest pain, and palpitations. None of them was initially suspected for EF. Regarding echocardiographic findings, predominant left ventricular involvement was identified, followed by bi-ventricular involvement. All the patients presented a restrictive filling pattern with severe left atrial dilation. In a retrospective analysis, Mocumbi criteria for diagnosis of EF were met in 100% of the patients, majority with moderate severity (77.8%). Cardiac MRI showed biventricular systolic function and volumes preserved. Focal subendocardial late gadolinium enhancement was observed on the apex and apical thrombus was confirmed in 66% of the patients. Conclusion: FE is an uncommon restrictive cardiomyopathy limited to tropical countries. Most of patients in our series presented isolated involvement of left ventricle, followed by bi-ventricular involvement, with ventricular function usually preserved. [ABSTRACT FROM AUTHOR]

The long-term results of transatrial transpulmonary tetralogy of Fallot (TOF) repair have been excellent. Progressive pulmonary regurgitation and consequent right ventricular (RV) dilatation are the most common long-term sequel of definitive repair in childhood. Overt systemic venous congestion after TOF repair is limited to the rare setting where RV dysfunction sets in due to deferred surgery or progressive arrhythmia. Here, we report a unique case of right heart failure from an unexpected etiology, 28 years after TOF repair. Cardiac catheterization confirmed findings of elevated right heart pressures. Magnetic resonance imaging showed obliteration of the RV apex with late gadolinium enhancement of the right ventricular apical endomyocardium. [ABSTRACT FROM AUTHOR]