Loeffler's Endocarditis Research Articles

Rezidivierende transitorische ischämische Attacken bei einer Patientin mit Pansinusitis

A 31-year-old woman presented with neurological deficits after an operation for sinusitis. The cranial MRI revealed multiple ischaemic lesions. Laboratory results showed a hypereosinophilia as well as elevated creatine kinase and troponin levels. The ECG implied ST elevations, the left ventricular ejection fraction was highly reduced and the cardiac MRI was suspicious for endomyocarditis. The cardiac biopsy demonstrated the findings of Loeffler's endocarditis. In conclusion the diagnosis of hypereosinophilic syndrome was made and identified as the cause of the neurological deficits.

Heart muscle disease

<h2>Abstract</h2> Primary cardiomyopathies are heart muscle diseases intrinsic to the myocardium. This group includes dilated cardiomyopathy (DCM), arrhythmogenic cardiomyopathy (ARVC), hypertrophic cardiomyopathy (HCM), restrictive cardiomyopathy (RCM) and unclassified cardiomyopathy. The cardiomyopathies may be classified pathophysiologically and display unique pathological and clinical features. ARVC is characterized by fibroadipose substitution of right ventricular myocardium and a high risk of sudden cardiac death. HCM may be symmetrical or asymmetrical and histologically is associated with widespread myocyte disarray. The clinical presentation and phenotype varies according to which type of myofibrillary gene mutation is responsible for disease. For example, mutations in troponin T carry a poor prognosis and a high risk of sudden cardiac death, whilst mutations in myosin binding protein C are associated with mild disease and onset in middle-age or late adult life. DCM is characterized by an increase in ventricular mass and cavity dilatation with or without thrombi in the atrial appendages. The histological features of DCM are often non-specific but myofibrillary loss, when present, is a helpful diagnostic feature. DCM is the commonest type of cardiomyopathy and most cases are idiopathic. RCM is the least common type of cardiomyopathy and includes diseases that ‘stiffen' the myocardium by fibrosis or infiltration. This group includes Loeffler's endocarditis, tropical endomyocardial fibrosis as well as cardiac amyloidosis and storage disorders. The inflammatory cardiomyopathies include myocarditis, idiopathic giant cell myocarditis and sarcoidosis. Myocarditis has an acute onset and is often accompanied by febrile illness, whereas sarcoidosis has an insidious onset and often presents with heart block.

Ventricular remodeling in Loeffler endocarditis: Implications for therapeutic decision making

Little is known about the morphological and functional evolution of ventricular abnormalities in Loeffler endocarditis. We describe 5 patients, including 3 with long-term echocardiographic follow-up, in whom apical obliteration due to fibro-thrombotic thickening of the endocardium showed favorable patterns of evolution. In one patient there was almost complete resolution of the obliterative process with consequently increased effective ventricular volume. In two patients formation of a flow-passage in the fibrocalcific apical 'floor' between the main medioventricular cavity and the apical chamber, leading to a 'double-chambered' left ventricle was observed. Medical therapy and appropriate anticoagulation, can induce favorable long-term ventricular remodeling in Loeffler endocarditis.

Left Ventricular Endocardial Calcification in a Patient with Myeloproliferative Disease

The case of a 57-year-old male with a history significant for myeloproliferative disease, chronic renal failure, hypertension, and prostate cancer is described. His complete blood count was remarkable for neutrophilia and, notably, eosinophilia. Subsequent to two syncopal episodes, a transthoracic echocardiogram was performed as part of the workup, which showed an unusual calcified mass in the left ventricular apical region but separate from the apical myocardium, with normal left ventricular systolic function. A transesophageal echocardiogram and computed tomography of the chest confirmed the presence of extensive calcification in the left ventricle of unusual location and shape. This patient probably had Loeffler endocarditis related to myeloproliferative disorder, complicated by calcification of the endocardial sclerotic lesions.

Endomyocardial fibrosis in Churg–Strauss syndrome assessed by cardiac magnetic resonance imaging

Cardiac involvement frequently occurs in hypereosinophilic syndrome. The endocardium seems to be most susceptible to disturbances deriving from eosinophilia as seen in Loeffler's endocarditis. Hypereosinophilia and cardiac involvement are also seen in Churg–Strauss syndrome. The present report deals with a patient with Churg–Strauss syndrome who exhibited marked endomyocardial fibrosis that was visualized by cardiac magnetic resonance imaging. Loeffler's endocarditis is the classical endocardial manifestation of hypereosinophilic syndrome. Similarities between Loeffler and Churg–Strauss syndromes had been described previously. Potentially, endocardial involvement is more common even in Churg–Strauss syndrome than expected so far. The significance of these findings remains to be shown.

An Autopsy Case of Loeffler's Endocarditis Presenting a Marked Reduction in Both Ventricular Cavities

An Autopsy Case of Loeffler's Endocarditis Presenting a Marked Reduction in Both Ventricular Cavities

Pathologic quiz case: a young woman with eosinophilia and heart failure. Primary hypereosinophilic syndrome with loeffler endocarditis.

Pathologic quiz case: a young woman with eosinophilia and heart failure. Primary hypereosinophilic syndrome with loeffler endocarditis.

Rapid Reversion of Loeffler's Endocarditis by Imatinib in Early Stage Clonal Hypereosinophilic Syndrome

Endomyocardial fibrosis (Loeffler's endocarditis) is the main cause of poor outcome in Hyper Eosinophilic Syndrome (HES) and Eosinophilic Leukemia (EL). Reversion of the cardiac damage has been seldom reported, and thrombi can superimpose on infiltrated walls, originating oembolic complications. The tyrosine kynase inhibitor imatinib has been recently employed in patients affected by HES or EL, with impressive results. We have treated with imatinib a young patient affected by Loeffler's endocarditis during EL. Loeffler's endocarditis was studied by transthoracic Doppler echocardiography with and without the contrast agent SonoVue. Cytogenetics, FISH and molecular analysis showed the presence of the FIP1L1/PDGFRA fusion gene, recently detected in a majority of HES patients. Standard echocardiography revealed a large infiltration of the apical region, with apparently pedunculate corpora floating in the LV chamber; after SonoVue injection, a thick endo-myocardial infiltration involving papillary muscles and tendinous chords appeared, which simulated mobile thrombi at standard echography. Treatment with low dose imatinib caused rapid regression of both eosinophilic proliferation and endomyocardiopathy. The fusion gene FIP1L1-PDGFRA was found significantly decreased after a few months of treatment. Using a contrast echocardiographic approach, we demonstrated the non-thrombotic origin of the "in plus" image in our patient and its rapid resolution following imatinib treatment. Imatinib is an excellent candidate for first line treatment of Loeffler's endocarditis, especially when the FIP1L1/PDGFA fusion gene is detected.

Chronic myelocytic leukemia with eosinophilia, t(9;12)(q34;p13), and ETV6-ABL gene rearrangement: case report and review of the literature

Chronic myelocytic leukemia (CML) is a chronic myeloproliferative disorder characterized by cytogenetic or molecular evidence of Philadelphia (Ph) chromosome, t(9;22)(q34;q11). Mild to moderate eosinophilia is commonly seen in CML. However, eosinophilia as a dominant feature of CML is extremely rare. We describe a case of Ph − CML with eosinophilia. Loeffler endocarditis, and t(9;12)(q34;p13) that resulted in an ETV6-ABL gene rearrangement/fusion identified to the best of our knowledge, for the first time by using commercially available fluorescence in situ hybridization probes.

A Case of Loeffler's Endocarditis with Acute Obstruction of Common Iliac Artery

A Case of Loeffler's Endocarditis with Acute Obstruction of Common Iliac Artery

Surgical pathology of primary cardiac and pericardial tumors.

Retrospective study of surgical pathology experience on cardiac and pericardial tumors at the University of Padua in the era of immunohistochemistry and endomyocardial biopsy. In the period 1970-1995, we studied 125 bioptic primary neoplasms: specimens were obtained from surgical resection in 116 cases, heart transplantation in 3, pericardiectomy in 3, endomyocardial biopsy in 2 and thoracoscopic biopsy in 1. Tumor histotype was established by light microscopy and more recently by immuno-histochemistry, using a large panel of antibodies, through avidin-biotin peroxidase method, against factor VIII-related antigen, ulex-europaeus, desmin, myoglobin, muscle-specific actin, smooth muscle-specific actin, vimentin, cytokeratins, leukocytic common antigen, neurofilaments and S100-protein. One hundred and thirteen were benign neoplasms: myxoma was the most frequent (87 cases) followed by pericardial cyst (8), endocardial papilloma (5), fibroma (3), rhabdomyoma (3), hematic cyst (2), teratoma (2), hemangioma (1), celothelioma (1) and lipoma (1). Malignancy was diagnosed in 12 cases, and consisted of pericardial mesothelioma (3), myxosarcoma (3), angiosarcoma (2), fibrosarcoma (2) and leiomyosarcoma (2); 4 of them were intracavitary atrial masses and were supposed to be atrial myxoma on the clinical ground. Differential diagnosis included endocardial thrombosis (10), metastasis of concealed extracardiac tumors (5), echinococcosis (3), and Loeffler's fibroplastic endocarditis (3). In 4 cases, cardiac mass histotype was defined without thoracotomy, through endomyocardial (3) and thoracoscopic (1) biopsy. A large spectrum of cardiac tumors is observed in the surgical pathology practice. Although the diagnosis of cardiac masses is easily attainable by routine imaging techniques, differential diagnosis between primary and secondary tumors, malignant and benign forms, and non neoplastic masses, is achievable only by a thorough microscopic study of surgical resections. The role of the cardiac pathologist is becoming crucial as in other fields of oncology.

Endocardial endothelial dysfunctionand heart failure

Like vascular endothelium, the EE plays a role in transendothelial transport, in coagulant and thrombotic processes, and in interactions with inflammatory cells. In addition, EE is involved in the modulation of cardiac performance of subjacent myocardium. EE dysfunction includes insufficient as well as excessive performance of any of its multiple functions. Dysfunction can progress from a disturbed modulation of myocardial performance and an imbalance in the release of growth factors to changes in EE cytoskeletal organization, with concomitant changes in transendothelial permeability, and in extreme cases, to loss of endothelial integrity and frank denudation. Structural and functional impairment of EE and of endocardial interstitial cells may be primary or secondary to the disease. Mechanical stress, various hormones and cytokines can initiate EE dysfunction. EE dysfunction may influence the development of cardiac failure in endo(myo)cardial fibrosis (Loeffler's endocarditis and carcinoid syndrome) and in dilated cardiomyopathy. Although Bouillaud, in 1836, was referring to endocarditis when stating: (quote: see text) his statement may presently find a much broader field of applicability in cardiology.

Loffler's Endocarditis

Figure 1. Loffler's Endocarditis. A transverse slice of the heart from a 65-year-old man with Loffler's endocarditis shows large mural thrombi (T) surrounding the thickened endocardium in both ventricles. Loffler's endocarditis is characterized by hypereosinophilia and endocardial thickening, which typically causes restrictive cardiomyopathy.

Eosinophil cationic granule proteins impair thrombomodulin function. A potential mechanism for thromboembolism in hypereosinophilic heart disease.

Thromboembolism is a prominent but poorly understood feature of eosinophilic, or Loeffler's endocarditis. Eosinophil (EO) specific granule proteins, in particular major basic protein (MBP), accumulate on endocardial surfaces in the course of this disease. We hypothesized that these unusually cationic proteins promote thrombosis by binding to the anionic endothelial protein thrombomodulin (TM) and impairing its anticoagulant activities. We find that MBP potently (IC50 of 1-2 microM) inhibits the capacity of endothelial cell surface TM to generate the natural anticoagulant activated protein C (APC). MBP also inhibits APC generation by purified soluble rabbit TM with an IC50 of 100 nM without altering its apparent Kd for thrombin or Km for protein C. This inhibition is reversed by polyanions such as chondroitin sulfate E and heparin. A TM polypeptide fragment comprising the extracellular domain that includes its naturally occurring anionic glycosaminoglycan (GAG) moiety (TMD-105) is strongly inhibited by MBP, whereas its counterpart lacking the GAG moiety (TMD-75) is not. MBP also curtails the capacity of TMD-105 but not TMD-75 to prolong the thrombin clotting time. Thus, EO cationic proteins potently inhibit anticoagulant activities of the glycosylated form of TM, thereby suggesting a potential mechanism for thromboembolism in hypereosinophilic heart disease.

Granulocyte-macrophage colony-stimulating factor-related eosinophilia and Loeffler's endocarditis [letter

Granulocyte-macrophage colony-stimulating factor-related eosinophilia and Loeffler's endocarditis [letter

Granulocyte-Macrophage Colony-Stimulating Factor-Related Eosinophilia And Loefflers Endocarditis

Granulocyte-Macrophage Colony-Stimulating Factor-Related Eosinophilia And Loefflers Endocarditis

Cardiac Localization of Eosinophil-Granule Major Basic Protein in Acute Necrotizing Myocarditis

THE association of eosinophilia and endomyocarditis has been well documented. A form of Loffler's endocarditis,1 characterized by eosinophilic pancarditis, muscle necrosis, and arteritis, is usually a feature of certain systemic diseases, including the hypereosinophilic syndrome,2 3 4 eosinophilic leukemia,5 polyarteritis nodosa,6 , 7 drug-induced hypersensitivity,8 bronchial asthma,9 10 11 biliary tract carcinoma,12 and parasitic disease.13 The cardiac involvement is characterized by a subacute course and symptoms of congestive heart failure. Treatment with glucocorticoids and cytotoxic agents is effective.14 Fulminant acute necrotizing eosinophilic myocarditis occurring in the absence of notable extracardiac disease has been reported only rarely.15 The disease has a short course and is rapidly fatal. . . .

Hypereosinophilic Syndrome with a Rapidly Fatal Course in a Saudi Child

Hypereosinophilic Syndrome with a Rapidly Fatal Course in a Saudi Child

Renal lesions in the obliterative cardiomyopathies: endomyocardial fibrosis and Loffler's endocarditis.

Electron and, or light microscopic examination of paraffin embedded renal tissue obtained from necropsies on eleven patients with obliterative cardiomyopathy showed a variety of abnormalities. Glomerular lesions present in patients with endomyocardial fibrosis included capillary wall thickening, basement membrane duplication, mesangial expansion and interposition, intraluminal fibrin and dense subendothelial deposits. These changes, some of which have been reported earlier in patients with Loffler's endocarditis, probably result from deposition and organisation of immune complexes.

Surgical treatment of Loffler's eosinophilic endocarditis.

Surgical treatment of Loffler's eosinophilic endocarditis.