Left Ventricular Endomyocardial Fibrosis Research Articles

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

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. 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. 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. 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.

Left Ventricular Thrombus in Patients with COVID-19 – A Case Series

Scarred culprit vessel territory secondary to nonreperfused myocardial infarctions (MIs), nonischemic cardiomyopathy, left ventricular (LV) noncompaction, endomyocardial fibrosis, and long-standing arrythmias are usually causes of LV thrombus (LVT) formation. However, in the setting of MI, large infarctions, apical akinesia or dyskinesia, LV aneurysms are often predisposed t'o the formation of LVT. The hypercoagulable or inflammatory disorder can rarely predispose to the formation of LVT. In early prethrombolytic and thrombolytic periods, LVT was present in 20%–50% of patients in the context of acute MI, more commonly in acute anterior or apical MI. While the incidence of LVT has dropped in recent times, its identification is expected to rise during the COVID-19 pandemic. Patients with chest pain are more likely to delay initial hospitalization because of a fear of contracting COVID-19. Infection with COVID-19 was associated with the remarkably hypercoagulable state which increased the risk of the early development of LVT in the setting of MI or underlying prethrombotic conditions. We present a series of four cases in which COVID-19 and cardiovascular disease were characterized by various configurations of large LVT.

Accessory left ventricular chamber in a cat: multimodality imaging description by cardiac magnetic resonance imaging and echocardiography

A left ventricular accessory chamber is a complex and uncommon phenotype consisting in a subdivision of the left ventricle by a thick-walled muscle bundle or septum into two cavities. Multiple aetiologies such as congenital double-chambered left ventricle and acquired endomyocardial form of restrictive cardiomyopathy have been reported.The endomyocardial form of restrictive cardiomyopathy, owing to its structural heterogeneity, can present a similar phenotype to the congenital abnormality with intraventricular lesions bridging the ventricular septum and left ventricular free wall. Conventional echocardiography is the first-line, accepted, non-invasive imaging modality to investigate underlying cardiac disease but presents limitations for the overall assessment of myocardial tissue. This report describes the use of cardiac magnetic resonance imaging as an additional imaging modality to provide multiplanar morphological, structural, and functional information of the myocardium. In the presented images, hyperintensities on cardiac magnetic resonance imaging within the myocardium along with hyperechoic and heterogeneous myocardial tissue on echocardiography and elevated troponin I were suggestive of a presumptive acquired pathological process such as feline endomyocarditis–left ventricular endomyocardial fibrosis complex, rather than a sole congenital anomaly. Although the diagnosis was not confirmed, this case exemplifies the advantages of using complementary multimodality imaging in a cat presented with a left ventricular accessory chamber.

Surgical resection of restrictive left ventricular endomyocardial fibrosis

Surgical resection of restrictive left ventricular endomyocardial fibrosis

Feline Panleukopenia Virus Is Not Associated With Myocarditis or Endomyocardial Restrictive Cardiomyopathy in Cats.

Canine parvovirus-2 (CPV-2) is nearly indistinguishable from feline panleukopenia virus (FPV) and is a well-known cause of viral myocarditis in young puppies; however, it is not known whether either FPV or CPV-2 naturally infects feline cardiomyocytes and causes myocarditis. Endomyocarditis (EMC) and left ventricular endomyocardial fibrosis (LVEF), clinically known as "endomyocardial restrictive cardiomyopathy," are important feline heart diseases suspected to have an infectious etiology. A continuum is suggested with EMC representing the acute reaction to an unknown infectious agent and LVEF the chronic manifestation of repair. The purpose of this study was to determine (1) whether there is natural parvovirus infection of the feline myocardium and (2) whether parvoviral infection is associated with feline EMC and/or LVEF. In a retrospective study, polymerase chain reaction and sequencing for the parvovirus VP1/2 gene was performed on archived heart tissue from cats with endomyocardial disease and controls. Similar methods were used prospectively on myocardial tissues from shelter-source kittens. Although 8 of 36 (22%; 95% confidence interval [CI], 11%-40%) shelter kittens had parvoviral DNA in myocardial tissue, VP1/2 DNA was not detected in 33 adult cases or 34 controls (95% CI, 0% to ∼11%). These findings were confirmed by in situ hybridization: adult cats did not have detectable parvovirus DNA, although rare intranuclear signal was confirmed in 7 of 8 shelter-source kittens. In kittens, parvovirus was not significantly associated with myocarditis, and in situ hybridization signal did not colocalize with inflammation. Although infection of cardiomyocytes was demonstrated in kittens, these data do not support a role for parvovirus in EMC-LVEF.

Endomyocardial fibrosis with right ventricular aneurysm mimicking ARVC – A case report from India

A 48-year-old man presented with chronic right sided heart failure. 2D echocardiography revealed the classical features of left ventricular endomyocardial fibrosis with a prominent right ventricular apical aneurysm. Right ventriculography further defined the aneurysm. Cardiac magnetic resonance images revealed a thin-walled, apical aneurysm of right ventricle with multiple septations and marked obliteration of left ventricular apex. A delayed-enhancement sequence after the infusion of contrast, demonstrated a hypersignal in the subendocardium, consistent with the right ventricular involvement of endomyocardial fibrosis. This patient had classical features of left ventricular endomyocardial fibrosis, while on the right side the typical features were missing. This aneurysm may be a passing phase of the natural history of endomyocardial fibrosis before the development of burned out stage. This aneurysm may later develop thrombus, and which may progress to fibrosis and apical obliteration. Endomyocardial fibrosis with right ventricular aneurysm has not heretofore been reported in the medical literature.

Doughnut appearance of left ventricle in isolated left ventricular endomyocardial fibrosis

A 55-year-old female presented with worsening dyspnea secondary to left ventricular endomyocardial fibrosis (EMF). Obliteration of left ventricular apex and hypertrophied papillary muscle gave the classical appearance of a “doughnut” on left ventricular angiography.We present here a novel doughnut appearance of left ventricle on angiogram.

Left Ventricular “Horseshoe-Thrombus”—A Case Report

Thrombi represent the most frequently found intracardiac masses. Left ventricular thrombus (LVT) is an important complication in patients with ischemic heart diseases and in those with dilated cardiomyopathy and systolic heart failure. The diagnosis of left ventricular thrombus remains important since anticoagulation will reduce the risk of systemic embolization and stroke. Despite advances in other imaging modalities, echocardiography remains the most important tool for diagnosis and risk stratification in patients predisposed to develop left ventricular thrombi. Mural thrombi formed on the left ventricular endocardium and horseshoe-shaped in chronic dilated cardiomyopathy, masquerading as left ventricular endomyocardial fibrosis was diagnosed by transthoracic 2D echocardiographic imaging in a 38 years old middle aged man. Background of this case highlighted the pathophysiology, high risk echocardiographic features and the role of anticoagulant therapy in LV thrombus with dilated cardiomyopathy.

Giant cell and granulomatous myocarditis necessitating cardiac transplantation: clinical, gross, and histopathological findings

Giant cell myocarditis and granulomatous myocarditis are rare diseases, leading to severe heart symptoms like tachyarrhythmia or atrial fibrillation with frequently fatal outcome. Typically, diagnosis is confirmed at necropsy or in explanted hearts, as the majority of cases remain clinically unrecognized. Explanted heart specimens of 355 heart transplant recipients receiving cardiac transplantation between January 1994 and December 2011 were examined. Unexpected granulomatous myocarditis was found in the explanted hearts of three patients and giant cell myocarditis in four patients. We investigated preoperative and postoperative course and gross and histopathological findings of these two different disorders, which were thought to be the same disease for a long time. Preoperatively, both groups suffered from mild to severe symptoms of heart failure. Gross findings of resected heart specimens showed right and left ventricular hypertrophy. Histological examination revealed a left ventricular endomyocardial fibrosis and inflammation infiltrates including giant cells with the presence or absence of granulomas. All patients with granulomatous myocarditis are alive. Three patients suffering from giant cell myocarditis showed a complicated postoperative clinical course, one heart transplant recipient developed Guillain–Barre syndrome due to cytomegalovirus reactivation 6 months after heart transplantation, another patient died due to cerebral edema shortly after transplantation, and another one died because of myocardial and cerebral infarction after tricuspid valve replacement. In consideration of the limitation of this study that a small number of patients were included, our results suggest that patients suffering from granulomatous myocarditis are older at heart transplantation and have a more favorable outcome compared with heart transplant recipients with giant cell myocarditis.

Non-tropical endomyocardial fibrosis by cardiovascular magnetic resonance, demonstrating the value of CMR in restrictive cardiomyopathy

Non-tropical non-hypereosinophilic left ventricular endomyocardial fibrosis (EMF) is a rare condition, not associated with defined substrates, about which there is a paucity of data in the literature. We describe a series of EMF patients presenting to our institution over 4 years in a high-volume CMR service.

The medical treatment of endomyocardial fibrosis in 2009

ObjectivesTo study the natural history of medically treated patients with endomyocardial fibrosis (EMF) and to see whether the usage of common drugs affected survival.DesignA real-world all-comers analysis with follow-up.SettingA teaching...

Endomyocardial Fibrosis in an Adult Mimicking Left Ventricular Mass

Endomyocardial fibrosis is a rare condition that occurs primarily in tropical countries. It can often mimick a variety of other common cardiac conditions such as apical hypertrophic cardiomyopathy and Ebstein anomaly. We report a case of a left ventricular mass that at histologic examination was found to be endomyocardial fibrosis.

Massively Calcified Left Ventricular Endomyocardial Fibrosis Occurring in a Caucasian Patient

A man, living in France, complained about dyspnea and weakness for the last year. At admission, the electrocardiogram showed atrial fibrillation. Transthoracic echocardiographyandcardiacmagnetic resonance imaging revealed a rare case of massively calcified left ventricular endomyocardial fibrosis (EMF), resulting in an obliteration of the apex (Fig. 1A and B). Despite the severity of the calcification intruding the fibrosis, h

Successful Reoperation After 17 Years in a Case of Endomyocardial Fibrosis

Endomyocardial fibrosis is an uncommon cause of congestive cardiac failure characterized by severe diffuse thickening of the endocardium, and the overall long-term prognosis of this disease is generally considered poor. We report a case of endomyocardial fibrosis that was initially treated by endocardial resection and mitral valve replacement, which was regularly followed-up. The patient underwent successful repair of a paravalvular leak after a period of 17 years. The treatment of left ventricular endomyocardial fibrosis with associated mitral valve disease by endocardial resection and mitral valve replacement is a good surgical option that provides good long-term results.

Angina-Like Chest Pain and Syncope as the Clinical Presentation of Left Ventricular Endomyocardial Fibrosis

A 47-year-old woman complained of angina-like chest pain, near-syncope, and syncopal episodes of 17 years' duration. Physical examination was unremarkable. A 12-lead resting ECG showed symmetrically inverted T waves in the inferior and anterolateral leads. A graded treadmill exercise stress test precipitated angina-like chest pain accompanied by a near-syncopal episode associated with a systemic arterial pressure of 60/40 mm Hg. Echocardiography disclosed left ventricular apical obliteration. Left ventriculogram showed a typical "ace of heart'' shadow as well as filling defects and apical obliteration. Endomyocardial biopsy of the left ventricle diagnosed left ventricular endomyocardial fibrosis. Thus, angina-like chest pain and near-syncopal episodes should be added to the list of clinical manifestations of pure left ventricular endomyocardial fibrosis.

Quantitative assessment of left ventricular regional wall motion in endomyocardial fibrosis

To analyze left ventricular (LV) regional wall motion in patients with endomyocardial fibrosis (EMF). The study comprised 88 patients, 59 of the female sex, with a mean age of 39+/-13 years (range, 9 to 65) and with echocardiographic and angiographic evidence of left ventricular EMF. The intensity of fibrous tissue buildup on contrast cineventriculography was classified as mild, moderate, or severe. The overall left ventricular ejection fraction (LVEF) was determined by using the area-length method on ventriculography. The motion was measured in 100 equidistant chords perpendicular to the centerline drawn in the middle of the final diastolic and systolic contours and normalized to cardiac size. Five left ventricular segments were analyzed: A--apical; AL--anterolateral; AB--anterobasal; IA--inferoapical; IB--inferobasal. Abnormality was expressed in units of standard deviation of the mean motion in a normal population of reference, comprised of 103 patients with normal LV according to clinical and electrocardiographic data, and angiographic standards. Mean LVEF was 0.47+/-0.12. Fibrous tissue buildup in the left ventricle was mild in 12 patients, moderate in 40, and severe in 36. The regions with the poorest ventricular wall motion were A (-1.4+/-1.6 standard deviation/chords) and IA (-1.6+/-1.8 standard deviation/chords) compared with that in AB (-0.3+/-1.9 standard deviation/chords), AL (-0.5+/-1.8 standard deviation/chords) and IB (-0.9+/-1.3 standard deviation/chords). No relation was observed between the intensity of fibrous tissue buildup and regional ventricular wall motion. A change in LV regional wall motion exists in EMF, and it is independent of the intensity of fibrous tissue buildup qualitatively assessed. Nonuniform involvement of the LV should be considered when planning surgery for this disease.

Visualization of Endomyocardial Fibrosis by Delayed-Enhancement Magnetic Resonance Imaging

An 18-year-old Venezuelan woman, with a history of idiopathic restrictive cardiomyopathy, presented at our hospital with ongoing dyspnea, palpitations, and lightheadedness. A medical evaluation revealed heart failure, pulmonary hypertension, and heparin-induced thrombocytopenia. Cardiac catheterization revealed normal coronary arteries. The left ventriculogram demonstrated late filling of the apex, which was almost obliterated by prominent trabeculations. Moderate mitral regurgitation with significant enlargement of the left atrium was observed. The ejection fraction was 44%, without regional wall motion abnormalities (Figure 1). Figure 1. End-systolic (A) and end-diastolic (B) frames of the left ventriculography show partial obliteration of the cavity …

Assessment of diastolic function in endomyocardial fibrosis: value of flow propagation velocity.

Endomyocardial fibrosis is manifested mainly by diastolic heart failure. However, diastolic function has not been well characterized in this disease. The aim of this study was to characterize left ventricular (LV) diastolic function in endomyocardial fibrosis (EMF) by echocardiography. Eighteen patients with LV EMF and 18 healthy subjects were studied. Cardiac volumes and ejection fraction were assessed by Simpson's method. Pulsed-wave Doppler was used to obtain mitral and pulmonary venous flow velocities and grade diastolic function. Pulsed-wave tissue Doppler imaging velocities along the septal side of mitral annulus, flow propagation velocity (Vp) of the early diastolic mitral inflow, and myocardial performance index were obtained. According to this grading method, four patients with normal diastolic function, five with impaired relaxation, five with pseudonormal, and four with a restrictive pattern were found. A positive correlation of these diastolic function grades and NYHA functional class was found (r = 0.66, P = 0.003). By "stepwise" logistic regression the best index that discriminated EMF patients from controls was Vp. The probability of occurrence of EMF = exp(7.9288 - 0.1366 Vp)/1 + exp(7.9288 - 0.1366 Vp). A wide range of diastolic function grades is found in patients with EMF and these correlated with functional class. Delayed myocardium relaxation, as reflected by altered Vp, was a frequent finding, making Vp the most useful index to discriminate EMF patients.

New approach for complete endocardiectomy in left ventricular endomyocardial fibrosis

J Thorac Cardiovasc Surg 2003;125:40-2

Mitral valve repair in patients with endomyocardial fibrosis

Between 1987 and 1990, 12 patients were operated on for endomyocardial fibrosis at our institution. Nine were treated by endocardectomy and mitral valve repair and constitute the material of this study. Ages ranged from 9 to 58 years (mean age, 32.5 years). Biventricular involvement was present in 3 cases, and 6 patients had predominantly left ventricular endomyocardial fibrosis. Six patients were in New York Heart Association class III/IV. Six patients had severe mitral insufficiency (3 to 4 / 4 ) and 3 patients had moderate mitral insufficiency ( 2 / 4 ). The operation consisted of left ventricular endocardectomy with complete detachment and mobilization of the posterior leaflet of the mitral valve in all cases. An autologous pericardial patch was used to reconstruct posterior leaflet continuity in 4 patients. In 7 patients a prosthetic mitral ring was used. In 3 patients right ventricular endocardectomy and tricuspid valve repair were also performed. All patients survived and none required early reoperation. Follow-up was complete and ranged from 9 to 38 months. No late deaths occurred, and 1 patient required mitral valve replacement for recurrent mitral regurgitation. Doppler echocardiographic studies were performed in 7 patients and revealed no or mild mitral insufficiency. In conclusion, mitral valve repair is safe and offers good early and late results in patients with endomyocardial fibrosis.