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.

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.

Ocupação apical por endomiocardiofibrose associada a trombo diagnosticada com ecocardiografia de contraste e resolvida com tratamento clínico / Apical occupation by endomyocardiofibrosis associated with thrombus diagnosed with contrast echocardiography and resolved with clinical treatment

A endomiocardiofibrose é uma doença negligenciada e predominante em países subdesenvolvidos. Apesar de sua frequência, ainda é considerada rara. Assim, a doença é marcada pelos seguintes achados: distorção arquitetural, alterações no enchimento ventricular e alterações da mobilidade segmentar. Ela afetando não só a dinâmica diastólica e sistólica, como também as funções das válvulas cardíacas. Em uma condição mais avançada da doença, pode haver formação de trombos apicais. Neste relato de caso, revisitamos a endomiocardiofibrose, apresentando um caso desafiador de paciente do sexo feminino de 52 anos, com clínica de insuficiência cardíaca classe funcional IV da New York Heart Association. A eletrocardiografia de repouso revelou ritmo sinusal com sobrecarga do ventrículo esquerdo com vetores de alta voltagem, infradesnivelamento retificado do segmento ST e onda T negativa em região anterolateral, podendo ser compatível com padrão de strain. Já na avaliação de imagens ecocardiográficas, revelou dilatação acentuada do átrio esquerdo, com ventrículos sem dilatações e imagem de ocupação apical hiperrefringente no interior do ventrículo esquerdo, sugerindo grande trombo séssil sobreposto à capa fibrosa endocárdica. Embora a conduta cirúrgica seja a mais apoiada em literatura em tais situações, a paciente aqui apresentada recebeu tratamento com anticoagulação oral por 1 mês e 24 dias. Ela evoluiu clinicamente bem, com melhora da classe funcional da New York Heart Association e, em ecocardiografia realizada 2 meses após o início da anticoagulação oral, houve demonstração da regressão da imagem de trombo apical, apoiada com a técnica de contraste endocavitário.(AU)

Endomyocardial fibrosis (EMF) is a neglected but prevalent disease in underdeveloped countries. Despite its frequency, it is still considered a rare disease. It is marked by the following findings: architectural distortion, ventricular filling changes, and segmental mobility changes affecting not only diastolic and systolic dynamics but also heart valve function. Apical thrombi can be formed in more advanced disease. In this case report, we revisit EMF and present the challenging case of a 52-year-old woman with New York Heart Association (NYHA) functional classification (FC) IV heart failure. Resting electrocardiography revealed sinus rhythm with left ventricular (LV) overload, high voltage vectors, rectified ST-segment depression, and a negative T-wave in the anterolateral region compatible with the strain pattern. The evaluation of echocardiographic images showed marked left atrial dilation, no ventricular dilatation, and hyper-refringent apical occupation within the LV suggestive of a large sessile thrombus superimposed on the endocardial fibrous layer. Although surgery is the most supported approach in the literature in such situations, this patient was treated with oral anticoagulants (OAC) for 1 month and 24 days. The patient progressed well with an improved NYHA FC. Endocavitary contrast echocardiography performed 2 months after OAC initiation showed regression of the apical thrombus image. (AU)

Early detection of myocardial dysfunction in a cat that gradually progressed to endomyocardial form of restrictive cardiomyopathy.

BACKGROUND: Restrictive cardiomyopathy (RCM) is a common myocardial disease in cats, characterized by diastolic dysfunction and atrial enlargement without myocardial hypertrophy. Especially, endomyocardial form of RCM, one of the subtypes in RCM, is characterized by endocardial fibrosis, endocardial scar bridging the interventricular septum and left ventricular (LV) free wall, and deformation and distortion of the LV. However, it is unclear how the myocardial dysfunction and the endocardial scar contribute to the pathophysiology of RCM disease progression. CASE PRESENTATION: A 3 years and 2 months old, intact male, Domestic shorthaired cat was presented for consultation of cardiac murmur. At the first visit (day 0), the notable abnormal finding was echocardiography-derived chordae tendineae-like structure bridging the interventricular septum and the LV free wall, resulting high-speed blood flow in the left ventricle. Electrocardiography, thoracic radiography and noninvasive blood pressure measurements were normal. No left atrial enlargement was observed, and LV inflow velocity showed an abnormal relaxation pattern. Although there was no abnormality in tissue Doppler imaging-derived myocardial velocity, two-dimensional speckle tracking echocardiography (2D-STE) revealed a decrease in the LV longitudinal strain and an increase in endocardial to epicardial ratio of the LV circumferential strain on day 0. On day 468, obvious left atrium enlargement and smoke like echo in the left atrium were observed. The LV inflow velocity was fused, and the tissue Doppler imaging-derived early-diastolic myocardial velocity of the septal mitral annulus decreased. Regarding 2D-STE, LV circumferential strain was further decreased, and right ventricular strain was additionally decreased. Although the general condition was good, we made a clinical diagnosis of endomyocardial RCM based on the above findings. On day 503, the cat showed the radiographic evidence of pulmonary edema and congestive heart failure signs. CONCLUSIONS: Cats with abnormal LV structure and associated myocardial dysfunction like this case needs careful observation. Additionally, 2D-STE indices may be useful for early detection of myocardial dysfunction in feline RCM.

Structural and functional cardiac changes in endomyocardial fibrosis treated with endomyocardial resection: Disease progression captured by multimodality imaging.

Eosinophilic myocarditis, a rare and under-recognized disease process, occurs due to cytotoxic inflammation of the endomyocardium that over time may lead to a restrictive cardiomyopathy. We report clinical, multimodality imaging, and pathologic findings in a 45-year-old woman over a 17-month period as she progressed from suspected acute eosinophilic myocarditis to phenotypic endomyocardial fibrosis resulting in recurrent ascites. Interval echocardiograms demonstrate definitive pathologic structural changes that reflect the hemodynamic consequences of the underlying cardiomyopathy. Despite a negative myocardial biopsy, characteristic findings on cardiovascular magnetic resonance imaging clarified the diagnosis which led to successful treatment with endomyocardial resection and valve replacements.

Detection of a rare cause of pulmonary hypertension by multimodality imaging: Left ventricular endomyocardial fibrosis.

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.

Cardiac magnetic resonance in arrhythmogenic cardiomyopathies.

Over the past few years, the approach to the 'arrhythmic patient' has profoundly changed. An early clinical presentation of arrhythmia is often accompanied by non-specific symptoms and followed by inconclusive electrocardiographic findings. In this scenario, cardiac magnetic resonance (CMR) has been established as a clinical tool of fundamental importance for a correct prognostic stratification of the arrhythmic patient. This technique provides a high-spatial-resolution tomographic evaluation of the heart, which allows studying accurately the ventricular volumes, identifying even segmental kinetic anomalies and properly detecting diffuse or focal tissue alterations through an excellent tissue characterization, while depicting different patterns of fibrosis distribution, myocardial edema or fatty substitution. Through these capabilities, CMR has a pivotal role for the adequate management of the arrhythmic patient, allowing the identification of those phenotypic manifestations characteristic of structural heart diseases. Therefore, CMR provides valuable information to reclassify the patient within the wide spectrum of potentially arrhythmogenic heart diseases, the definition of which remains the major determinants for both an adequate treatment and a poor prognosis. The purpose of this review study was to focus on the role of CMR in the evaluation of the main cardiac clinical entities associated with arrhythmogenic phenomena and to present a brief debate on the main pathophysiological mechanisms involved in the arrhythmogenesis process.

Where is the right ventricle? Accurate diagnosis with cardiovascular multimodality imaging.

Endomyocardial fibrosis (EMF) is a globally unattended disease with significant rates of morbidity and mortality. It has a higher prevalence in tropical and subtropical countries compared to the rest of the world. Endomyocardial fibrosis can affect the atrioventricular valves, along with all four chambers of the heart, but spares the myocardium. Patients currently undergo symptomatic treatment with diuretics and vasodilators to enhance quality of life, although medical therapy alone is associated with poor prognosis. Hence, patients with severe symptoms prefer surgical treatment. Modern multimodality imaging, however, can help these definitions to be made more accurately.

Endomyocardial fibrosis: past, present, and future.

Endomyocardial fibrosis (EMF) is a neglected idiopathic disorder, predominant in tropical and subtropical regions of the developing world. It is characterized by fibrotic thickening of the endocardium and myocardium of one or both ventricles. EMF was an important cause of heart failure which accounted for up to 20% of the cases in endemic areas of Africa (rural community in Mozambique), but during the last few years, incidents of the disease have decreased considerably. Although its pathogenesis and etiology are not fully understood, its pathology resembles conditions such as eosinophilic cardiomyopathy and hypereosinophilic syndrome. Extensive fibrosis of the ventricular endocardium causing architectural distortion, impaired filling, and valvular insufficiency defines the disease. Confined to peculiar and limited geographical areas, the etiology remains blurred and it carries a grim prognosis. Medical care currently remains very challenging as one-third to half of patients with an advanced disease die within 2 years. Surgery in the correct setting can increase survival and especially in patients with advanced heart failure.

Right ventricular involvement in hypertrophic cardiomyopathy: Patterns and implications.

Although hypertrophic cardiomyopathy (HCM) is the most common inherited cardiomyopathy worldwide, the criteria for its definition and most of the literature concern the left ventricle, thus confirming the theory that the right ventricle is the neglected one. Right ventricular (RV) involvement includes structural and functional changes with significant impact on clinical presentation and prognosis. The pattern of RV hypertrophy can be variable with possible dynamic obstruction. Histological findings suggest similar pathogenetic changes in both ventricles supporting the common myopathic process with sarcomeric mutations. Systolic dysfunction of the RV is subtle, and the classical echocardiographic indices are usually within normal limits, while global longitudinal strain is significantly impaired. Diastolic dysfunction of the RV is also evident in patients with HCM possibly due to fibrosis of the RV free wall and/or the obstruction of the RV filling with significant prognostic implications. RV involvement in HCM is associated with increased incidence of supraventricular and ventricular arrhythmias, severe dyspnea, pulmonary thromboembolism, progressive heart failure, and increased risk of sudden cardiac death. Therefore, the RV should be routinely included in the detailed assessment of patients with HCM.

A case presentation of patient from northern China with endomyocardial fibrosis.

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.

Apical hypertrophic cardiomyopathy with apical endomyocardial fibrosis and calcification: Two case reports.

RATIONALE: Apical hypertrophic cardiomyopathy (AHCM) is a rare form of hypertrophic cardiomyopathy which affects predominantly the apex of the left ventricle. Generally, left ventricular enlargement is not present in AHCM; additionally, endomyocardial fibrosis, and calcification are also rare. PATIENT CONCERNS: A 61-year-old female (Case 1) and a 60-year-old female (Case 2) both presented with the symptoms of atypical chest pain, dyspnoea, exercise intolerance, palpitations. DIAGNOSIS: Magnetic resonance and single-photon emission computed tomography (SPECT) revealed apical hypertrophic cardiomyopathy. Furthermore, 2D-transthoracic echocardiogram showed left atrium and ventricular enlargement, as well as endomyocardial fibrosis and calcification. Based on these findings, the patients were diagnosed with AHCM. INTERVENTIONS: Both the patients were treated with ACEI, metoprolol, and aspirin. Additionally, both these patient underwent genetic test. OUTCOMES: The results of the genetic test of the 2 cases for hypertrophic cardiomyopathy (HCM) were negative. However, the gene mutation for dilated cardiomyopathy (TMPO) was detected in one of the cases. No change in condition during follow-up. LESSONS: In past reports, Apical hypertrophic cardiomyopathy has been shown to have a benign prognosis. But in this case report, the imaging studies of the 2 patients suggest a poor prognosis. Furthermore, diagnosing cardiomyopathy should require multimodality imaging examinations to rule out differential diagnoses.