Diagnosis Of Endomyocardial Fibrosis Research Articles

Biventricular Endomyocardial Fibrosis associated with Ulcerative Colitis: a case report

Endomyocardial Fibrosis (EMF) is a restrictive cardiomyopathy characterized by the fibrotic involvement of the endomyocardium. Describe an uncommon case and the general aspects of this entity. A 13-year-old female patient was admitted with a suspect of acute gastroenteritis. Cursed with intense protein depletion and deteriorating health conditions. During the investigation was diagnosed with Ulcerative Colitis. The Doppler echocardiogram showed hyperrefingent images and a tumoration-sugestive lesion in both ventricles. The cardiac magnetic resonance confirmed the presence of tumorations in the apical portion of right and left ventricles. The patient was submitted to a biventricular resection of the masses. After the intervention, clinical therapy was started. The diagnosis of EMF was later confirmed on the histopathologic examination. The clinical presentation of EMF is unspecific and difficult to recognize, demanding deep investigation skills. The correlation with imaging exams for diagnosis is essential, especially the echocardiogram and the magnetic resonance. In this case, it is observed the association between Ulcerative Colitis and EMF, as well as the potential of the imaging modalities in the confirmation and therapeutic guidance of this pathology.

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.

Endomyocardial fibrosis in Sudan: clinical and echocardiographic features.

SummaryObjective:Endomyocardial fibrosis (EMF) is a rare disease and is often an underdiagnosed and forgotten cardiomyopathy. The objective of this study was to document the current frequency of EMF in Sudan by defining and selecting cases from patients attending the echocardiography laboratory. Additionally we aimed to create an EMF registry for Sudan.Methods:The study started in January 2007 and is on-going. All the patients attending our echocardiography clinics in four different hospitals in Khartoum, Sudan, were included. Transthoracic echocardiography was used as the main diagnostic and selection tool. The diagnosis of EMF was based on predefined criteria and definitions, and was further supported by additional clinical, ECG, laboratory and chest X-ray findings.Results:Out of 4 332 cases studied, 23 (0.5%) were found to have features of EMF. Females constituted 52% and the age range was 24 to 67 years. All patients presented with dyspnoea grades III–IV. Advanced heart failure with gross fluid overload was seen in 54% of cases and ascites was seen in 30%. EMF was biventricular in 53%, left ventricular in 29% and right ventricular in 18% of cases. Apical and ventricular wall fibrosis was found in all cases, followed by atrial enlargement, atrioventricular valve incompetence, ventricular cavity obliteration, restrictive flow pattern and pericardial effusion. Additional echocardiographic features are defined and discussed.Conclusion:Although a rare disease, cases of EMF can be identified in Sudan if a high index of suspicion is observed. New echocardiographic features of ventricular wall layering, endocardial fibrous shelf and endomyocardiopericarial fibrosis were identified and are discussed.

Endomyocardial Fibrosis: Echocardiographic Profile

Evolution of two-dimensional and color Doppler echocardiography identified the typical features of Endomyocardial Fibrosis (EMF) such as dense ventricular apical fibrosis, dysfunction of atrioventricular valve and sub-valvular apparatus and cavity dimensions. This is the tool used most for the diagnosis of EMF in areas where the disease is endemic in Africa. Today echocardiography is used as a screening tool to identify cases of EMF at the community level and it could be confirmed at the bed side. Background of these cases highlighted the echocardiographic features of EMF in different age groups and the oldest one reported at the age of 85 years in a female in advanced stage at this coastal district of Thoothukudi in India.

How to recognize endomyocardial fibrosis?

Endomyocardial fibrosis is a disease of unknown cause, characterized by the development of restrictive cardiomyopathy. Although it is endemic in Africa, some cases have been described in Asia, South America and Europe, where a substantial unfamiliarity with this disease still exists. Moreover, differential diagnosis of endomyocardial fibrosis with other cardiomyopathies can be difficult, especially in asymptomatic patients and in initial stage of the disease. After initial echocardiographic analysis, the gold standard imaging technique is the cardiac magnetic resonance. Adjunctive diagnostic tools as endomyocardial biopsy can be considered in ambiguous cases and can help in patient management.

Usefulness of cardiac MRI in the early diagnosis of endomyocardial fibrosis

Usefulness of cardiac MRI in the early diagnosis of endomyocardial fibrosis

A new typical finding in late gadolinium enhanced images for the diagnosis of endomyocardial fibrosis - the double V sign

Introduction Endomyocardial fibrosis (EMF) is a restrictive cardiomyopathy presenting with ventricular apical filling, possibly containing fibrotic tissue with thrombus and/or calcification. Late gadolinium enhancement (LGE) can detect apical fibrosis in EMF patients, as a hyper intense linear image usually in a letter “V” like shape pointing to the ventricular apex. Recently, we have observed a pattern of a double layer hyper and hypo intense in LGE images, also in a “V” like shape, possibly corresponding to fibrosis plus thrombus/calcification.

Complementary Roles of Transthoracic Two‐Dimensional Color Doppler Imaging and Myocardial Contrast Echocardiography in Diagnosis of Endomyocardial Fibrosis

Endomyocardial fibrosis (EMF) is a rare progressive restrictive cardiomyopathy of unknown etiology that mainly presents with heart failure. The presence of a small ventricle with obliteration of the apex and a large atrium are two-dimensional echocardiographic findings that are highly suggestive of EMF. Cardiac magnetic resonance with delayed enhancement allows detection of subendocardial fibrosis with good histopathological correlation, providing a noninvasive modality for diagnosing EMF. We herein report a case of EMF in which the complementary role of two-dimensional color Doppler imaging and myocardial contrast echocardiography proved to be useful in the diagnosis of this clinical entity, which was later confirmed by cardiac magnetic resonance, surgery, and histopathology.

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 …

Usefulness of Echocardiography and Doppler Echocardiography in Endomyocardial Fibrosis

Objective: Our goal was to demonstrate the usefulness of echocardiography and cardiac Doppler echocardiography (echo-Doppler) in the diagnosis of endomyocardial fibrosis, an unusual restrictive cardiomyopathy in Argentina. Methods: Between 1980 and 1998, we studied 10 women (aged 27 to 58 years) with endomyocardial fibrosis confirmed by surgery and/or endomyocardial biopsy. Of the 10 cases of endomyocardial fibrothrombosis, 8 were biventricular and 2 were left ventricular. Six patients had only an echocardiographic study, and the last 4 patients (after 1987) had an echo-Doppler study also; 3 had a transesophageal echocardiography examination as well. Seven patients had grade III-IV dyspnea, 2 had an edematous-ascitic syndrome, and 1 had right heart failure at the first examination. Four patients died of heart failure and 1 of overimposed sepsis. Surgery was successful in 2 patients with the biventricular form of the disease. In one of them, fibrotic decortication was performed in both ventricles together with tricuspid and mitral replacement. In the other, the right side was not surgically treated because of its mild engagement. One patient was lost to follow-up, and 3 patients are awaiting surgery at this writing. Results: In all 10 patients, echocardiography was the first diagnostic tool used. In M-mode echocardiography, the typical image showed the “square root” sign in the septum and posterior wall in addition to the “merlon” sign, characterized by a hypercontractile basal ventricle opposing an obliterated apex. In 2-dimensional echocardiography, inversion of the normal sized heart with obliterated ventricles and dilated atria were seen in the whole group. In 1 patient, the fibrous thrombus was limited to the apex of the right ventricle (Shaper’s type 1) in a biventricular form, whereas in the left side of this patient and in the other 9 patients, the fibrous thrombus that initially occupied the apex engaged the posterior papillary muscle, pulling the posterior valve downward (Shaper’s type 2) and generating tricuspid and/or mitral regurgitation that was always mild or moderate. The fibrous thrombus never altered the movement of the underlying myocardium. There were hypoechoic and hyperdense echoes inside the fibrotic material (the latter compatible with calcium), and in all 10 patients, different grades of pericardial effusion were found. Echo-Doppler showed the same minimal percentage of change in mitral and tricuspid velocities as found in healthy patients, which clearly differentiates endomyocardial fibrosis from constrictive pericarditis. Furthermore, a restrictive pattern was observed on both atrioventricular valves when both sides were engaged with a markedly short tricuspid deceleration time. Pulmonary veins showed a markedly diastolic D wave and a broad reversal A wave (the latter presented a low velocity when the wall of the left atrium was diseased) caused by an increased end-diastolic left ventricular pressure to the same extent throughout the respiratory cycle. Hepatic veins showed a markedly deep diastolic forward wave throughout the respiratory cycle and a marked reversal with inspiration. Conclusions: We showed (1) echocardiographic studies of a significant number of patients with this unusual disease, (2) the characteristic diagnostic signs in M-mode and 2-dimensional echocardiography, and (3) the common echo-Doppler patterns shared by all subjects studied with this technique. (J Am Soc Echocardiogr 2000;13:385-92.)