Endomyocardial Disease Research Articles

Pediatric restrictive Cardiomyopathy presenting with congestive heart failure: Rare case report and literature review

Restrictive cardiomyopathy (RCM) is an uncommon form of cardiomyopathy and is characterized by impaired diastolic filling of the ventricles due to increased myocardial stiffness and a normal ventricular systolic functions. In India, RCM is frequently observed in the young and endemic endomyocardial diseases are seen in a significant number of cases. RCM is the least common of all the cardiomyopathies among pediatric patients. RCM has a poor prognosis and commonly requires a cardiac transplant. We are reporting here a case of seven-year-old female child presenting with recurrent chest infections and congestive heart failure since early childhood.

Relative interventricular septal brightness for subtype diagnosis of cardiac amyloidosis: pixel brightness analysis using echocardiographic images

Abstract Background In the subtypes of cardiac amyloidosis (CA), transthyretin-related (ATTR) CA has been reported to exhibit a higher prevalence of microcalcifications in the interventricular septum (IVS) compared to other subtypes. Whether microcalcifications in the IVS can stratify CA subtypes remains unclear. Methods A total of 442 patients with CA: 76 light chain/ amyloid A (AL/AA) type; 255 wild type ATTR (ATTRwt) and 48 variant ATTR (ATTRv) and 19 patients with endomyocardial biopsy-confirmed hypertensive heart disease (HHD) were subjected to echocardiographic pixel brightness quantification of the IVS using open-access image analysis software. The IVS Brightness Index (IVSBI) was defined as the ratio of the average pixel brightness in the IVS to that in the left ventricular (LV) wall opposite the IVS (see figure 1). Results The IVSBI from apical 4-chamber view was higher in both the ATTRwt group (median 1.40, IQR 1.22 to 1.67) and the ATTRv group (1.59, 1.32 to 2.00) compared to the HHD group (1.20, 1.02 to 1.37) and the AL/AA group (1.25, 1.11 to 1.46), respectively (P <0.001, see figure 2). In the 273 CA patients undergoing myocardial technetium-99m-pyrophosphate scintigraphy, patients with positive results (1.41, 1.24 to 1.70) exhibited a higher IVSBI compared to those with negative (1.19, 1.09 to 1.46) (P = 0.006). In Cox proportional hazards analysis, age, gender and LV ejection fraction adjusted hazard ratio for the all-cause death of IVSBI (0.5 increase) was 2.0 (95% CI 1.2 to 3.5, P = 0.012) among ATTRwt patients (n=157). Conclusions An IVSBI obtained from the apical 4-chamber view could prove valuable in differentiating ATTR subtypes in CA, potentially revealing subtle septal calcifications. A prognostic value may be derived from the elevation of IVSBI in ATTRwt patients.figure 1figure 2

In-Depth Review of Loeffler Endocarditis: What Have We Learned?

Loeffler endocarditis, eosinophilic endocarditis or eosinophilic endomyocardial disease are conditions associated with hypereosinophilia and they affect the heart function. Loeffler endocarditis is a rare endomyocardial disorder thought to be caused by eosinophilic damage. The disorder is characterized by inflammatory infiltration, formation of thrombus within cardiovascular system, and ultimately fibrosis of the afflicted area. It can lead to multiple severe complications, including thromboembolic disease, thickening of fibrous tissue in the endocardium of ventricles, valve involvement, apical obliteration, and various heart disorders. Although early clinical intervention can lead to remission, the underlying mechanisms of the disorder remain unresolved. In the present article, we summarise the existing literature concerning Loeffler endocarditis based on PubMed, Web of Science, and other medical databases to conduct an in-depth review of the epidemiology, etiology, pathophysiological mechanisms, staging, diagnosis, treatment and prognosis of Loeffler endocarditis. Meanwhile, we provide novel patients data and clinical figures of Loeffler endocarditis to supplement the understanding of this cardiac disorder. The findings presented in this article provide a basis for further studies and can be used to improve management of the disorder.

Eosinophilic Myo-Pericarditis Presenting as Cardiac Tamponade

Eosinophilic myocarditis is a rare and acute life threatening condition often associated with drug reactions, eosinophilic granulomatous polyangiitis (formerly Churg-Strauss syndrome), Loeffler endomyocardial disease, parasite infections, and idiopathic hypereosinophilic syndrome. We describe a case of eosinophilic granulomatous polyangiitis presenting as progressive pericardial effusion and cardiac tamponade.

EOSINOPHILIC MYO-PERICARDITIS PRESENTING AS CARDIAC TEMPONADE

Eosinophilic myocarditis is a rare and acute life threatening condition often associated with drug reactions, eosinophilic granulomatous polyangiitis (formerly Churg-Strauss syndrome), Loeffler endomyocardial disease, parasite infections, and idiopathic hypereosinophilic syndrome. We describe a case

Endomyocardial diseases cardiomyopathy: A review and pooled analysis of pathophysiology, diagnosis and clinical management

Endomyocardial diseases cardiomyopathy: A review and pooled analysis of pathophysiology, diagnosis and clinical management

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.

Hypereosinophilic syndrome mimicking acute coronary syndrome

Hypereosinophilic syndrome (HES) is a heterogeneous group of disorders with peripheral blood hypereosinophilia and eosinophil-mediated organ involvement. It may be primary, secondary, or idiopathic. In very rare cases, HES can be familial occurring as an autosomal dominant disorder. Cardiac involvement usually presents as heart failure, intracardiac thrombus, arrhythmias, and rarely as acute coronary syndrome (ACS) and is a major cause of morbidity and mortality. Cardiac magnetic resonance imaging has emerged as a diagnostic modality in diagnosis of eosinophilic endomyocardial disease. We report a case of a young male with familial HES presenting as ACS and discuss diagnostic and therapeutic clinical management.

Development of eosinophilic endomyocardial disease in a patient with episodic angioedema and eosinophilia

Development of eosinophilic endomyocardial disease in a patient with episodic angioedema and eosinophilia

Central nervous system involvement of hypereosinophilic syndrome: A report of 10 cases and a literature review

BackgroundThe central nervous system (CNS) may be affected in those cases of peripheral eosinophila without a secondary cause (hypereosinophilic syndrome, HES). The pathomechanism of CNS involvement in HES remained uncertain. MethodsWe included those with CNS symptoms and peripheral eosinophilia (>1500/μl) at the time of presentation. Those with identifiable causes of eosinophilia were excluded. In addition to data analysis of our cases, we reviewed the literature using the Medline database. ResultsTen patients were recruited. Half of them presented with altered mental status. None of the patients had significant risk factors such as endomyocardial disease (EMD). Their brain magnetic resonance imaging (MRI) showed multiple ischemic lesions in the border zone (BZ) and other cortical areas. In addition to our 10 cases we included 67 cases from a literature review. The total 77 cases were systemically analyzed. We found that the most common type of CNS involvement was cerebrovascular disease (CVD, 63.6%), multifocal lesions in BZ were the most common feature on MRI (45.5%), many patients presented with altered mental status (40.3%), and direct eosinophil infiltration into brain parenchyma was rare in pathologic studies and not found in the patients with CVD. Furthermore, although EMD was one of the major cardiac abnormalities (26.0%), cardiac workup more frequently showed non-EMD or normal findings. Cardiac abnormalities, altered mental status, and higher eosinophil counts were more common in those with poor outcomes, whereas an isolated CNS manifestation was more frequent in those with favorable outcomes. Finally, the presence of altered mental status was the most significant predictor of poor clinical outcomes, whereas isolated CNS involvement and corticosteroid treatment were significant predictors for favorable outcomes. ConclusionsMultiple lesions on MRI were suggestive of an embolic mechanism, but diagnostic workup failed to find significant risk factors such as EMD in the most cases. Impaired anticoagulation by eosinophilic cationic proteins could be an alternative mechanism. Direct invasion of eosinophils into the CNS was rare and those cases with isolated CNS manifestations showed a better prognosis. The preferential indirect involvement of CNS by eosinophils (“CNS-tropism”) may underlie the neurological manifestations of HES. Early administration of corticosteroid after ruling out secondary causes can be beneficial in HES with CNS manifestation.

Eosinophilic endocardiomyopathy: a case presentation

Eosinophilic endocardiomyopathy is a major cause of morbidity and mortality among patients with hypereosinophilic syndrome (HES), a group of disorders characterized by the sustained overproduction of eosinophils, where eosinophilic infiltration causes the damage to multiple organs. We present the case of a 63-year-old man who had been investigated at the out-patient clinic for malaise, effort intolerance, dry cough and cognitive disturbances six months before his presentation to our hospital. He had been diagnosed with hypereosinophilia (51.14 x 10^9/L) and leukocytosis (69.7 x 10^9/L). Bone marrow biopsy resulted negative for abnormal myeloid maturation or lymphoproductive disorder. A brain magnetic resonance confirmed postischemic lesions and gastroscopy revealed esophageal varices grade I. According to immunologist's recommendation, who previously excluded systemic autoimmune disease, the patient was referred to our Cardiology Clinic for further investigations. The transthoracic echocardiography showed a dilated cardiomyopathy with global hypokinesia and ejection fraction of 40%. A multi-slice computer tomography coronarography (MSCT) excluded the presence of coronary artery disease. Cardiac magnetic resonance imaging demonstrated biventricular enlargement and global hypokinesia (trueFISP cine), myocardial oedema in the mid-cavity and in the apical segments (T2 STIR), diffuse endocardial late gadolinium enhancement of the left ventricular mid-cavity and apical segments and of almost the entire right ventricle (true FISP PSIR) indicating fibrosis/necrosis. Thrombi at both ventricular apices were also identified. These findings present the final stage in the time course of eosinophilic endomyocardial disease. The patient was dismissed with anticoagulation and immunosuppressive therapy. Two months later the patient was feeling better and the eosinophil counts significantly decreased. Eosinophilic endocardiomyopathy is characterized by three stages: an acute necrotic stage significant for direct endomyocardial infiltration and damage, an intermediate phase, in which thrombi form along the damaged endocardium and a fibrotic stage characterized by endomyocardial fibrosis. Cardiac magnetic resonance imaging is an important non-invasive tool for detection of all stages of eosinophil-mediated heart damage.

Eosinophilic myocarditis.

Eosinophilic myocarditis is caused by activation of eosinophilic granulocytes whereby there is a release of eosinophilic granules. Quite a few of the released compounds, especially eosinophilic cationic proteins, have a tissue-damaging effect also in the myocardium. Eosinophilia may be due to hypersensitivity, parasitic infection etc. However, in hypereosinophilic syndromes and Loeffler's endomyocardial disease, the eosinophilia is"idiopathic." I believe that a clinical spectrum of the disease exists that is intimately correlated with the number of eosinophils and especially the degree of activation. When more is known about the cardiotropism of eosinophils in the various clinical settings, an important step will have been taken toward both the understanding and the treatment of this disease spectrum.

Gadolinium-enhanced cardiovascular magnetic resonance for the detection and characterization of Loeffler endocarditis in patients with hypereosinophilic syndrome

Hypereosinophilic syndrome (HES) is a rare disorder characterized by a marked and persistent peripheral blood eosinophilia combined with organ-system dysfunction caused by eosinophil-associated tissue damage [ 1 Chusid M.J. Dale D.C. West B.C. Wolff S.M. The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature. Medicine (Baltimore). 1975; 54: 1-27 Crossref PubMed Scopus (1183) Google Scholar , 2 Klion A.D. Bochner B.S. Gleich G.J. et al. The Hypereosinophilic Syndromes Working G. Approaches to the treatment of hypereosinophilic syndromes: a workshop summary report. J Allergy Clin Immunol. 2006; 117 (Epub 2006 May 3.): 1292-1302 Abstract Full Text Full Text PDF PubMed Scopus (303) Google Scholar ]. The clinical manifestations of HES are variable and depend on the target-organ affection by eosinophils. Cardiac involvement, originally described by Loeffler [ [3] Löffler W. Endocarditis parietalis fibroplastica mit Bluteosinophilie. Ein eigenartiges Krankheitsbild. Schweiz Med Wochenschr. 1936; 66: 817-820 Google Scholar ], is frequent and cardiac dysfunction is the major cause of morbidity and mortality in HES-patients [ [4] Weller P.F. Bubley G.J. The idiopathic hypereosinophilic syndrome. Blood. 1994; 83: 2759-2779 PubMed Google Scholar ]. The cardiac pathology of HES has been temporally divided into three stages: early acute necrotic, then thrombotic, and finally a late fibrotic stage [ [4] Weller P.F. Bubley G.J. The idiopathic hypereosinophilic syndrome. Blood. 1994; 83: 2759-2779 PubMed Google Scholar ]. The diagnosis of eosinophilic cardiac disease is based on the co-existence of endomyocardial eosinophils on myocardial biopsy and cardiovascular abnormalities for which other possible causes have been excluded. However, biopsy is invasive and most standard non-invasive diagnostic tools such as echocardiography are often unsatisfactory in detecting cardiac involvement. Contrast-enhanced cardiovascular magnetic resonance imaging (CE-CMR) can characterize different myocardial tissue abnormalities by typical patterns of late gadolinium enhancement (LGE), including necrosis, inflammation, and fibrosis [ [5] Pennell D.J. Cardiovascular magnetic resonance. Circulation. 2010; 121: 692-705 Crossref PubMed Scopus (211) Google Scholar ]. Thus, CE-CMR is an attractive technique to directly visualize the various cardiac pathologies that occur during the different stages of Loeffler endocarditis. So far, the usage of CMR in diagnosing HES associated heart disease has been described only in single case reports [ 6 Puvaneswary M. Joshua F. Ratnarajah S. Idiopathic hypereosinophilic syndrome: magnetic resonance imaging findings in endomyocardial fibrosis. Australas Radiol. 2001; 45: 524-527 Crossref PubMed Scopus (25) Google Scholar , 7 Syed I.S. Martinez M.W. Feng D.L. Glockner J.F. Cardiac magnetic resonance imaging of eosinophilic endomyocardial disease. Int J Cardiol. 2008; 126 (Epub 2007): e50-e52 Abstract Full Text Full Text PDF PubMed Scopus (63) Google Scholar , 8 Cheung S.C. Chan C.W. Insights of prognostication of Davies disease: what could we learn from serial magnetic resonance imaging studies?. Int J Cardiol. 2009; 12: 12 Google Scholar , 9 Chang S.A. Kim H.K. Park E.A. Kim Y.J. Sohn D.W. Images in cardiovascular medicine. Loeffler endocarditis mimicking apical hypertrophic cardiomyopathy. Circulation. 2009; 120: 82-85 Crossref PubMed Scopus (13) Google Scholar , 10 Deb K. Djavidani B. Buchner S. et al. Time course of eosinophilic myocarditis visualized by CMR. J Cardiovasc Magn Reson. 2008; 10: 21 Crossref PubMed Scopus (55) Google Scholar ] but no systematic evaluation has been reported. The purpose of this study was to evaluate the presence of cardiac involvement in patients with HES using CMR.

Eosinophil Granule Protein Localization in Eosinophilic Endomyocardial Disease

In this case report, eosinophilic endomyocardial disease was diagnosed in a patient on the basis of immunohistochemical evidence of eosinophil granule protein deposition in the heart, even in the absence of eosinophilic infiltration on cardiac biopsy.

Echocardiographic features of cardiac amyloidosis presenting as endomyocardial disease in a 54-year-old male

Although primary cardiac amyloidosis is a rare affliction of the heart, its clinical and pathology features have been well described. Patients generally present with exertional dyspnea, chest discomfort, or congestive heart failure. Cardiac imaging typically reveals ventricular wall thickening, systolic and diastolic dysfunction, valvular thickening, and pericardial effusion. In the case reported herein, a patient with suspected cardiac amyloid demonstrated electrocardiographic and echocardiographic findings that were not only inconsistent with typical depositional patterns, but also potentially suggestive of endomyocardial disease.

Neglected tropical cardiomyopathies: II. Endomyocardial fibrosis

Endomyocardial fibrosis (EMF) was described as a distinct clinicopathological entity in 1948 in Uganda.w1 Initially several terms were used to describe the disease. These included tropical endomyocardial disease, endocarditis parietalis...

Loeffler endocarditis: What have we learned?

Loeffler endocarditis, eosinophilic endomyocardial disease or fibroplastic endocarditis appears to be a subcategory of the Hypereosinophilic syndrome in which the heart is predominantly involved. It is an uncommon myocardial disease, thought to be secondary to eosinophils damage, characterized by fibrous thickening of the endocardium of one or both ventricles, leading to apical obliteration and multiple cardiovascular complications. Despite all the efforts, the ultimate responsible mechanisms of this entity remain unresolved. Many theories have been raised trying to explain this phenomenon, but nowadays the enigma in relation to the different patterns of evolution continues. In this concise review we discuss the different pathophysiologic theories postulated and the management of the cardiovascular complications. Perhaps it will serve to assist in recognition of patients with the same condition around the world.

Cardiac magnetic resonance imaging of eosinophilic endomyocardial disease

Idiopathic hypereosinophilic syndrome (HES) is rare and frequently involves the heart with development of endomyocardial fibrosis, a characteristic restrictive cardiomyopathy. Non-invasive diagnosis is frequently challenging. Recent developments in cardiac magnetic resonance (CMR) imaging techniques offer potential for improved detection and characterization of this entity. We describe the use of contrast-enhanced CMR to demonstrate thrombus, endomyocardial fibrosis and inflammation in eosinophilic endomyocardial disease. A three-layered appearance consisting of normal myocardium, thickened fibrotic endocardium with inflammatory exudate, and overlying thrombus was seen on cine-images. Post-contrast perfusion and delayed enhancement images confirmed the presence and location of endomyocardial fibrosis and thrombus. CMR is an important and powerful addition to current non-invasive diagnostic tools for the clinical diagnosis of eosinophilic endomyocardial disease, and may potentially obviate the need for cardiac biopsy in the future.

FIP1L1–PDGFRA positive chronic eosinophilic leukemia in Tunisian patients

Hypereosinophilic syndromes (HES) are a heterogenous group of rare disorders characterized by sustained and otherwise unexplained overproduction of eosinophils with organ involvement and consecutive dysfunction. Detection of the FIP1L1-PDGFRA fusion gene or the corresponding cryptic 4q12 deletion in HES supports the diagnosis of chronic eosinophilic leukemia (CEL) and provides a molecular explanation for the pathogenesis of this disorder. We screened seven Tunisian patients fulfilling the WHO criteria of HES for the presence of the FIP1L1-PDGFRA fusion gene using nested reverse transcription polymerase chain reaction on peripheral blood samples. Four of the seven patients were positive for this fusion gene. Sequence analysis revealed a substantial heterogeneity of the fusion transcripts due to the involvement of several FIP1L1 exons. All patients were male. The median age at diagnosis was 24 years (range, 18-50); one patient had a history of hypereosinophilia of more than 10 years. Two patients had clinically important and symptomatic eosinophilic endomyocardial disease with thrombotic events. Splenomegaly was constant in FIP1L1-PDGFRA positive CEL but not in the other HES patients (only 1/3).

‘Hissing snake’ left ventricle thrombotic phase of hypereosinophilic endomyocardial disease

A 33-year-old girl with chronic allergic state and history of a recent travel in South-East Asiatic countries was admitted because of acute heart failure with pulmonary oedema. ECG revealed sinus tachycardia with diffuse T-wave abnormalities, whereas two-dimensional echocardiography in apical four-chamber view showed thickening of the mid-apical portion of left ventricular (LV) walls with almost complete apex obliteration …