Application of Routine Echocardiography Combined with Contrast-Enhanced Echocardiography in the Diagnosis of Endomyocardial Fibrosis.

BACKGROUND: In China, endomyocardial fibrosis (EMF) is a type of restrictive cardiomyopathy that is rare and easy to be misdiagnosed. Our aim was to examine the value of routine echocardiography (RE) combined with contrast-enhanced echocardiography (CEE) in EMF diagnosis. METHODS: We studied 16 EMF patients retrospectively, from 2012 to 2022. All patients underwent RE, from which 11 underwent CEE. We divided the patients into three groups: biventricular EMF (Bi-EMF), right ventricular EMF (RV-EMF), and left ventricular EMF (LV-EMF) based on different lesion locations. We also analyzed the clinical and conventional ultrasound characteristics of the three groups of patients and examined the ventricle opacification (VO) and myocardial contrast echocardiography (MCE) characteristics of patients who underwent CEE. RESULTS: All patients with EMF subtypes had the following ultrasound findings: apical occlusion on one or both sides, corresponding atrial dilatation, atrioventricular valve regurgitation in varying degrees, and cardiac diastolic dysfunction. Of the subjects, 69% had apical thrombus calcification and 81% had mild pericardial effusion. RV-EMF patients had statistically significant right atrial enlargement compared with the other two groups (p < 0.05), moderate or severe tricuspid regurgitation, and inferior vena cava (IVC) dilation. LV-EMF patients had statistically significant left atrial enlargement compared with the other two groups (p < 0.05), elevated pulmonary artery systolic pressure (PASP), and 60% of LV-EMF patients had moderate or severe mitral regurgitation. Bi-EMF patients had bilateral atrial enlargement, an IVC collapsibility index <50%, and elevated PASP. CEE was performed in 11 patients, whose ventricle opacification showed no contrast filling in the apical occluded area and the heart chambers presented the "mushroom sign" during diastole. Their myocardial contrast echocardiography (MCE) showed delayed perfusion in the thickened fibrotic endomyocardium (TFE) of the apical and subvalvular regions and perfusion defect in the apical thrombus. The number and location of thrombus determined by the MCE combined with RE were the same as those detected by cardiac magnetic resonance imaging (CMR). MCE shows that the position and range of TFE are similar to CMR. CONCLUSIONS: EMF has characteristic RE presentation, and different EMF subtypes have unique characteristics. CEE can better display heart structure, ventricular wall motion, and tissue perfusion compared to RE. A combination of RE and CEE can make the EMF diagnosis more accurate, thereby allowing early treatment for EMF patients.

Endomyocardial fibrosis with ischaemic stroke.

A Caucasian man in his mid-50s presented to the emergency department with sudden onset right-sided weakness and dysarthria. CT angiogram demonstrated a small volume of left middle cerebral artery territory infarction after which the patient underwent successful thrombolysis. Initial ECG showed diffuse T wave inversion. He was admitted to an acute stroke ward where 72 hours of telemetry demonstrated normal sinus rhythm without arrhythmia. Transthoracic echocardiogram showed a reduced ejection fraction of 47% in the absence of any regional wall motion abnormality. An echogenic mass was noted in the apical inferior septal wall. Cardiac MRI demonstrated circumferential mid to apical endocardial thickening with characteristic appearances consistent with endomyocardial fibrosis (EMF). Furthermore, a visible hypointensity in the apex confirmed left ventricular thrombus adherent to the area of EMF. The patient was started on anticoagulation and heart failure therapy. He has recovered from the ischaemic stroke and is currently under outpatient surveillance.

Endomyocardial fibrosis: a tragic conundrum or a mere negligence?

Often termed as 'vanishing mystery' epidemiologically, endomyocardial fibrosis (EMF) continues to be the the most common cause of restrictive cardiomyopathy in Africa and some parts of the Indian subcontinent, where it is considered a significant public health problem. On the other hand, in developed countries, it is considered a rare phenomenon. This entity is considered incurable due to lack of targeted therapies, even after 75 years since its discovery. The authors describe here a case of an Indian male, in his early 30s who presented with complaints of dyspnoea on minimal daily activities, swelling of both legs and abdominal fullness for 3 months. The clinical features were suggestive of isolated right heart failure. He was diagnosed with EMF on echocardiography and the findings were confirmed on cardiac MRI. The patient has progressed to develop biventricular dysfunction and is being managed symptomatically with diuretics due to unavailability of targeted treatment options at present.

Endomyocardial fibrosis and apical calcification: A case report with unusual presentations of apical hypertrophic cardiomyopathy.

RATIONALE: Apical hypertrophic cardiomyopathy (ApHCM) is a phenotypic variant of hypertrophic cardiomyopathy. Endomyocardial fibrosis and endocardial calcification are especially rare in ApHCM. PATIENT CONCERNS: The main symptoms was chest tightness, palpitation, shortness of breath, and fatigue. Echocardiography and imaging examinations found apical hypertrophy along with endocardial calcification and endomyocardial fibrosis. Abnormal structural changes led to thrombosis and made the left ventricle a flat shape resembling an "apple." DIAGNOSES: The typical presentations, hypertrophic apex on echocardiography and an elevated N-terminal pro-brain natriuretic peptide level indicated the diagnosis of ApHCM and heart failure with preserved ejection fraction. INTERVENTIONS: Optimal medical therapy including the administration of ApHCM, heart failure and atrial fibrillation to improve symptoms and life quality. OUTCOMES: Since discharge, the patient could perform normal daily activities and had no discomfort based on the optimal medical therapy. LESSONS: We report a ApHCM patients with unusual presentations of endomyocardial fibrosis and apical calcification. This case highlights the importance of understanding the specific pathological changes of ApHCM for treatment and prognosis.

Endomyocardial substrate of ventricular arrhythmias in patients with autoimmune rheumatic diseases.

INTRODUCTION: Delayed enhancement-magnetic resonance imaging (DE-MRI) has demonstrated that nonischemic cardiomyopathy is mainly characterized by intramural or epicardial fibrosis whereas global endomyocardial fibrosis suggests cardiac involvement in autoimmune rheumatic diseases or amyloidosis. Conduction disorders and sudden cardiac death are important manifestations of autoimmune rheumatic diseases with cardiac involvement but the substrates of ventricular arrhythmias in autoimmune rheumatic diseases have not been fully elucidated. METHODS AND RESULTS: 20 patients with autoimmune rheumatic diseases presenting with ventricular tachycardia (VT) (n = 11) or frequent ventricular extrasystoles (n = 9) underwent DE-MRI and/or endocardial electroanatomical mapping of the left ventricle (LV). Ten patients with autoimmune rheumatic diseases underwent VT ablation. Global endomyocardial fibrosis without myocardial thickening and unrelated to coronary territories was detected by DE-MRI or electroanatomical voltage mapping in 9 of 20 patients with autoimmune rheumatic diseases. In the other patients with autoimmune rheumatic diseases, limited regions of predominantly epicardial (n = 4) and intramyocardial (n = 5) fibrosis or only minimal fibrosis (n = 2) were found using DE-MRI. Endocardial low-amplitude diastolic potentials and pre-systolic Purkinje or fascicular potentials, mostly within fibrotic areas, were identified as the targets of successful VT ablation in 7 of 10 patients with autoimmune rheumatic diseases. CONCLUSION: Global endomyocardial fibrosis can be a tool to diagnose severe cardiac involvement in autoimmune rheumatic diseases and may serve as the substrate of ventricular arrhythmias in a substantial part of patients.

Left ventricle endomyocardial fibrosis: a case report.

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.

Complications of Endomyocardial Fibrosis and Their Physiological Compromise: A Review.

Endomyocardial fibrosis (EMF) is an endemic idiopathic cardiomyopathy denoted by restricted and fibrous myocardium and endocardium. Apical fibrosis may cause thickening of the myocardium and extend from the ventricular apices through to the valves, thereby, restricting inflow. EMF is a critical condition that is predominant in the young population. However, it may also be associated with older individuals. Identified areas recorded with cases include the under-developed populations, that is, the tropics of Africa- Uganda, Mozambique; South Asia, and South America. Unlike in the preceded years, further studies are currently on focus to completely evaluate this medical condition. Additionally, this review encompasses the complications that arise from EMF as studied and reported from case studies, clinical trials, experiments, and research including heart failure, atrioventricular valves regurgitations, arrhythmias, serous cavities effusions, circulatory shock, stroke, myocardial ischemic syndromes, hepatic, and end-organ dysfunctions. Complications like heart failure may be very fatal to its victims. Moreover, left ventricular thrombosis may probably impose systemic or cerebral thromboembolism, which would risk the probability of a stroke. All in all, suggestions in the management strategies that have proved effective and advances in cardiac transplantation and of recently proposed therapeutic targets equally take part of this review and may form a foundation to diminishing the remaining inch of EMF and its outcomes.

Endomyocardial fibrosis related sudden cardiac death; two autopsied case-reports from Egypt.

Endomyocardial fibrosis (EMF) is an idiopathic tropical disorder that is characterized by the development of restrictive cardiomyopathy. Neglected EMF can cause sudden cardiac death (SCD) in adults. Conclusive diagnosis of EMF depends on autopsy after death. In an effort to attract the interest of the community for this rare disease, we report two cases of SCD that were diagnosed as EMF during autopsy in Egypt. Both cases were thoroughly investigated with emphasis on death circumstances and post-mortem anatomical and histopathological findings. The two cases were for adult males presented with SCD following a quarrel with a negative medical history and family history regarding cardiac diseases. No trauma or drug abuse. The autopsy revealed hypertrophied hearts, thick fibrosed endocardium, patchy myocardial fibrosis, and filling of the apex by fibrosis and calcifications. In one of them, there was a huge mural thrombus reaching the level of the mitral valve that totally occluded the cavity of the left ventricle. Histopathologically, fibrosis was confirmed, and no eosinophils were detected. In contrast to previously reported cases in Egypt, the left ventricle was solely affected. Despite the rarity of the disease outside the tropics, the frequency of EMF cases is more likely to be more than the number of reported cases. EMF should be considered as possible cause of SCD during autopsy. Further studies are needed to clarify the etiology and epidemiology of EMF.

Exploration of the Mechanism of Tripterygium Wilfordii in the Treatment of Myocardial Fibrosis Based on Network Pharmacology and Molecular Docking.

BACKGROUND: A network pharmacology study on the biological action of Tripterygium wilfordii on myocardial fibrosis (MF). METHODS: The effective components and potential targets of tripterygium wilfordii were screened from the TCMSP database to develop a combination target network. A protein-protein interaction network was constructed by analyzing the interaction between tripterygium wilfordii and MF; then, the Gene Ontology (GO) classification and Kyoto Encyclopedia of Genes and Genomes (KEGG) enrichment analysis was performed. Furthermore, molecular docking was utilized to verify the network analysis results. RESULTS: It was predicted that MF has 29 components contributing to its effectiveness and 87 potential targets. It is predicted that Tripterygium wilfordii has 29 active components and 87 potential targets for the treatment of MF. The principal active components of tripterygium wilfordii include kaempferol, ß-sitosterol, triptolide, and Nobiletin. Signaling pathways: AGE-RAGE, PI3K-Akt, and MAPK may be involved in the mechanism of its action.7 Seven key targets (TNF, STAT3, AKT1, TP53, VEGFA, CASP3, STAT1) are possibly involved in treating MF by tripterygium wilfordii. CONCLUSION: This study shows the complex network relationship between multiple components, targets, and pathways of Tripterygium wilfordii in treating MF.

A rare case of sudden death due to endomyocardial fibrosis in Italy: A differential diagnosis with other causes of restrictive cardiomyopathy.

A 45-years-old Indonesian woman was admitted to the hospital with nausea, vomiting, abdominal pain and tachyarrhythmia. Atrial fibrillation was found at ECG, blood tests showed mild hepatic function alterations. Radiological exams showed bilateral pleural effusions, ascites, hepatomegaly. Systolic and diastolic functions of the left ventricle were found to be strongly compromised at US. Physical conditions and laboratory results worsened rapidly, followed by multi organ failure. Death occurred 28 hours after admission. An autopsy was performed to clarify the cause of death and investigated medical malpractice. External examination showed jaundice skin and at internal examination bilateral pleural and pericardial effusions, ascites, mild cardiomegaly, ventricular endocardial fibrosis, a thrombus in tight junction to the left ventricular wall and hepatic necrosis were observed. Histological investigations revealed a massive endomyocardial fibrosis, detected through Azan-Mallory and Verhoef-Van-Gieson stain, and confirmed the presence of hepatic and renal necrosis. Toxicological and microbiological investigations were negative. The cause of death was a global cardiac dysfunction caused by a restrictive cardiomyopathy in an Indonesian woman affected by an undiagnosed and asymptomatic endomyocardial fibrosis. In this case, autopsy and histopathological investigations were fundamental to diagnose an occult endomyocardial fibrosis, which is an idiopathic disorder of tropical and subtropical regions of the world. The not common incidence of this disease in our country and its unusual clinical onset were at first perceived as a medical malpractice from the relatives. Consequently, the clinical aspects of the case intertwine with the medicolegal implications concerning the undiagnosed disease and the causality with the patient's death.

Fibrosis Endomiocárdica: Presentación de Caso Clínico y Revisión de la literatura / Endomyocardial Fibrosis: Case Report and Literature Review

La fibrosis endomiocárdica (FEM) es una enfermedad rara y potencialmente letal caracterizada por la infiltración de tejido fibrótico en el endocardio y miocardio. Es más comúnmente observada en regiones tropicales y subtropicales, y se cree que es causada por la combinación de factores genéticos y ambientales. Su patogénesis aún no está del todo clara, pero es posible que esté relacionada con una respuesta inmune anormal ante múltiples estímulos, incluyendo infecciones virales, parásitos, trastornos hematológicos y deficiencias alimentarias. Esta condición es una de las principales manifestaciones de los síndromes hipereosinofílicos (SHE). Pese a los avances en el manejo, el pronóstico de los pacientes con FEM persiste siendo pobre, con un alto riesgo de recurrencia y de progresión hacia insuficiencia cardiaca. A continuación, presentaremos el caso de un paciente joven con un cuadro de insuficiencia cardiaca de predominio derecho en el contexto de un síndrome hipereosinofílico de base, en quien se establece el diagnóstico de FEM según lo documentado en las imágenes cardiacas.

Endomyocardial Fibrosis: Case Report and Literature Review. Endomyocardial fibrosis (EMF) is a rare and potentially life-threatening cardiac disorder characterized by the infiltration of fibrotic tissue into the endocardium and myocardium of the heart. It is most seen in tropical and subtropical regions, and it is believed to be caused by a combination of environmental and genetic factors. The exact pathogenesis of EMF is not fully understood, but it is thought to be related to an abnormal immune response to various stimuli, including viral infections, parasites, and dietary deficiencies. This condition is one of the main manifestations of hypereosinophilic syndromes. Despite advances in treatment, the prognosis for patients with EMF remains poor, with a high risk of recurrence and progression to heart failure. We present a case of a young male patient with an episode of right heart failure in the context of a chronic hypereosinophilic syndrome in which the diagnosis of EMF is made with the aid of cardiovascular imaging.

Surgical Management of The Endomyocardial Fibrosis of Right Ventricle Mimicking Tumor with Recurrent Pulmonary Embolism.

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.

Intracardiac mass revealing a rare form of cardiomyopathy.

We present the case of a woman with a 2-month history of exertional dyspnoea and fatigue in which echocardiography revealed a cavity-obliterating right ventricular mass. Further imaging evaluation using cardiac magnetic resonance showed a thrombotic mass as well as diffuse myocardial oedema and endomyocardial fibrosis (EMF) that involved both ventricles. In the absence of any other cause (including peripheral eosinophilia), the diagnosis of idiopathic EMF was established. This case highlights this uncommon disease in non-tropical areas.

Diagnostic Imaging Analysis and Care of Patients with Endomyocardial Fibrosis Based on Wireless Network Smart Medical Application.

The heart is one of the most important organs of the human body, but in recent years heart disease has become one of the human health killers and this paper explores endomyocardial fibrosis, which is a common cardiomyopathy, commonly seen in infants and children, and refers to a diffuse elastic fibrous disease of the endocardium. The purpose of this paper is to explore the diagnostic imaging analysis and care of patients with endocardial heart machine fibrosis using wireless network intelligent medical technology, aiming to provide a new power basis for the treatment of the disease in related patients. This paper proposes a new endocardial segmentation algorithm that aims to process image information using image features, intervene in image noise reduction and smoothing, etc., and use image grayscale values to confirm cardiac cavity grayscale values as a basis for physicians to make certain judgments for the diagnosis of patients with endocardial machine fibrosis. The experimental results show that the atrial fibrillation group is distinctly higher compared to the sinus rhythm group, with values remaining between 25 and 39, which is a significant advantage compared to other methods.

Hypereosinophilic syndrome with cardiac infiltration and congestive heart failure in a cat.

Hypereosinophilic syndrome is an uncommon disorder in the cat. It is a heterogeneous group of conditions defined by a persistent hypereosinophilia associated with organ damage directly attributable to tissue hypereosinophilia. A seven-year-old castrated domestic shorthair cat presented to the emergency service for dyspnea. Initial physical examination identified the presence of a grade III/VI systolic left parasternal murmur with no gallop or arrhythmia. A snap N-terminal-pro hormone brain natriuretic peptide was abnormal, and a point-of-care ultrasound revealed mild pleural effusion, scant pericardial effusion, and an enlarged left atrium. There was leukemia (72.35 K/uL, reference range 4.5-15.7 K/uL) predominated by eosinophilia (33.84 K/uL; reference range 0-1.9 K/uL). On echocardiogram, there was concentric hypertrophy of the left ventricular walls with irregular endocardial borders. The left atrium was enlarged with evidence of spontaneous echogenic contrast. The mitral valve was thickened with a vegetative lesion on the anterior leaflet. Despite treatment, the patient experienced cardiopulmonary arrest, and cardiopulmonary resuscitation was unsuccessful. Complete necropsy with histopathology revealed eosinophilic infiltrates in multiple organs and the presence of a severe, acute-on-chronic, fibrinous, and eosinophilic-granulomatous endomyocarditis with mural thrombosis and marked endocardial fibrosis. This case represents an unusual presentation of the hypereosinophilic syndrome in the cat with cardiac involvement and congestive heart failure as a primary clinical sign.

An unusual tropical endomyocardial fibrosis: a case report.

Tropical endomyocardial fibrosis (TEF) is a rare condition that occurs primarily in tropical countries, leading to a severe heart failure with heart restrictive filling patterns. Eosinophilia appears to be a trigger leading to the development of the disease; thus, numerous etiologic factors accompanied by eosinophilia have been postulated, although none have been confirmed. The massively calcified form of TEF is exceptional and easily diagnosed by multimodal imaging; but it is a very rare condition with high surgical challenge. the best prevention remains the testing and treatment of parasitic infections frequently encountered in these countries.

[Cardiac imaging in infiltrative cardiomyopathies. What cardiovascular imaging modalities to propose in hypertrophic cardiomyopathies ?] / Rôle de l'imagerie cardiaque dans les cardiomyopathies infiltratives: Quels examens d'imagerie demander devant une hypertrophie ventriculaire gauche ?

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.