Recurrent prosthetic valve dysfunction in a patient with idiopathic hypereosinophilic syndrome: A case report and literature review.

Idiopathic hypereosinophilic syndrome with cardiac involvement is characterized by endocardial fibrosis and thrombosis. Here, we report a case of mitral valve prosthetic dysfunction in a patient with idiopathic hypereosinophilic syndrome and review related cases in the literature. Valve replacement with a 27-mm St. Jude bioprosthetic mitral valve improved his symptoms and hypereosinophilia. A 4-year follow-up revealed that the prosthetic valve was intact without thrombosis. Because mechanical prosthesis implantation yields poor surgical outcomes, bioprosthesis is the preferred choice for patients with idiopathic hypereosinophilic syndrome. Medications for controlling eosinophilia may improve the long-term outcomes of valve replacement surgeries.

Morphologically proved ANCA positive Loeffler's pancarditis: medical and surgical treatment.

Loeffler's endocarditis remains is a very rare disease, develops due to eosinophilic inflammation predominantly of the endocardium with an outcome in fibrosis and massive thrombus formation and. He is generally characterized by an unfavorable prognosis. Clinical case of a 42-year-old patient with Loeffler endocarditis is presented. The development of the disease was preceded by a polyvalent allergy, mild dry eye syndrome and pansinusitis with a single eosinophilia of blood up to 16%. The reason for the hospitalization was the appearance of biventricular heart failure. During the previous year, the level of blood eosinophils remained normal, a threefold increase in the level of eosinophilic cationic protein was observed once. A 20-fold increase in the pANCA level, a 2.5-fold increase in the level of antibodies to DNA, an antibody to the nuclei of cardiomyocytes 1:160 were detected. The diagnosis was made on the basis of electrocardiography data (low QRS voltage, atrial hypertrophy), echocardiography, multispiral computed tomography and magnetic resonance imaging of the heart (thickening and delayed contrasting of the endocardium, massive thrombosis of the left ventricular apex with obliteration of its cavity, encapsulated fluid in the pericardium with compression of the right ventricle). Systolic dysfunction, severe signs of restriction and arrhythmias were absent. Trombectomy, tricuspid valve plasty, pericardial resection, suturing of an open oval window were performed. Signs of active inflammation with single eosinophils, vasculitis, perimuscular sclerosis, endocardial sclerosis were detected in morphological and immunohistochemical studies of endo-, myo-, pericardium. Viral genome was not found. The therapy with methylprednisolone 24 mg/day, azathioprine 75 mg/day was started. Six months after the operation, the symptoms of heart failure are completely absent, the thrombosis did not recur.

Mitral valve replacement in a patient with idiopathic hypereosinophilic syndrome and pulmonary arterial hypertension.

BACKGROUND AND AIMS: Idiopathic hypereosinophilic syndrome, a rarely seen systemic disease, may cause cardiac valvular lesions by eosinophilic infiltration. This report describes management of a 25-year-old woman with idiopathic hypereosinophilic syndrome, severe mitral stenosis, and pulmonary arterial hypertension. METHODS: The patient was presented with haemoptysia and dyspnea on exertion. Echocardiography showed severe mitral stenosis and pulmonary arterial hypertension. RESULTS: After hematological stabilization, she underwent mitral valve replacement using a No. 27 bovine pericardial valve. In the intensive care unit she had a pulmonary hypertensive crisis, which ameliorated gradually with sedation and nitroglycerin. She was extubated and discharged on the second and seventh days, respectively. CONCLUSION: Surgical experience for the patients with mitral dysfunction caused by idiopathic hypereosinophilic syndrome is limited. When mitral valve replacement is needed, the ideal type of prosthesis remains unclear and the presence of pulmonary arterial hypertension further complicates the management. We think that bioprosthetic valves would be the appropriate choice in eosinophilic mitral dysfunction requiring valve replacement.

Tricuspid valve relocation with endomyocardial fibrosis removal for Löeffler's endocarditis.

Löffler endocarditis with hypereosinophilic syndrome is rare but can cause critical ventricular obliteration by endomyocardial fibrosis. A 52-year-old woman experienced severe right heart failure with extreme shrinkage of her right ventricle, severe tricuspid regurgitation and marked right atrial enlargement. Preoperative tests showed identical pressures in the right atrium and pulmonary artery. Endocardial stripping was done, and to enlarge the right ventricle, we relocated the anterior and posterior tricuspid leaflets cephalad, up the right atrium wall, to 'ventricularize' a portion of the right atrium, with autologous pericardial augmentation of the tricuspid leaflets. An annuloplasty ring was added to reinforce the relocated tricuspid attachment. Right heart pressures normalized postoperatively. The patient recovered uneventfully. She has received corticosteroid therapy continuously and has shown no recurrence of heart failure in the 5 years since surgery.

Heart transplantation in a patient with endomyocardial fibrosis due to hypereosinophilic syndrome.

Idiopathic hypereosinophilic syndrome (HES), a systemic disease that commonly involves the heart leading to progressive endomyocardial fibrosis, frequently manifests as restrictive cardiomyopathy. In this report, we describe the first case of a patient with endomyocardial fibrosis due to HES who underwent orthotopic heart transplantation at our institution. A literature review and discussion are included.

[Acute idiopathic hypereosinophilic syndrome in a rottweiler]. / Syndrome d'hyperéosinophilie idiopathique aigu chez un rottweiler.

The idiopathic hypereosinophilic syndrome is a part of the yet rather unknown diseases and the aetiology remains at the least hypothetical. This syndrome is characterized by a variable hypereosinophilemia as well as a massive infiltration of several organs by mature eosinophils, causing an important tissue damage leading to organ dysfunctions and resulting in the patient death. In this reported case, the acute idiopathic hypereosinophilic syndrome was diagnosed in a 4-year rottweiler female dog based on an increased eosinophilemia and the infiltration of stomach, small intestine, colon, pancreas, spleen, ganglions, skin, lungs and bone marrow by mature eosinophiles. The acute development of the disease precluded any therapeutical hope success.

Atypical presentation of eosinophilic endomyocardial disease.

Idiopathic hypereosinophllic syndrome is classically defined as prolonged, unexplained peripheral eosinophilia in a patient presenting with evidence of end-organ damage. The heart is frequently Involved, resulting In eosinophilic endomyocardial disease and eventually restrictive cardlomyopathy. The mortality rate is high because of progressive heart failure or ventricular arrhythmias. We describe a patient who presented with a left ventricular apical thrombus without notable peripheral eosinophilia. Findings from clinical evaluation and extensive diagnostic testing, including right ventricular biopsy, were Inconclusive. Resection of the thrombus and subjacent endomyocardium revealed eosinophilic Infiltration of the endomyocardium, which led to the diagnosis of eosinophilic endomyocardial disease. Clinicians should be aware of the variable presentation of patients with eosinophil-associated endomyocardial disease so that affected patients may benefit from early diagnosis and treatment.

Response of idiopathic hypereosinophilic syndrome to treatment with imatinib mesylate.

Idiopathic hypereosinophilic syndrome (HES) is a rare hematologic disorder characterized by persistent eosinophilia with organ involvement. Patients with HES have a poor prognosis, but the disease course can be heterogeneous. Treatment of HES has included corticosteroids, chemotherapeutic agents such as cyclophosphamide, vincristine, hydroxyrea, and most recently interferon-alpha (IFN-alpha) which has shown long-term beneficial effects. We herein report on a patient with HES who had disease resistant to steroids, and chemotherapy with 2-chlorodeoxyadenosine and cytarabine, but who had a significant response after only 8 days of treatment with imatinib mesylate 100mg daily. The possible mechanism of response is discussed. This observation may lead to a better understanding of the pathophysiology of HES, and may provide a new form of effective therapy for the disease.

Recurrent thrombosis of prosthetic mitral valve in idiopathic hypereosinophilic syndrome.

A 41-year old man, with idiopathic hypereosinophilic syndrome, had severe mitral regurgitation. He had two subsequent mechanical mitral valve thromboses in the setting of rising eosinophilia. A Carpentier-Edwards bioprosthesis was used for the third valve replacement. Some features of myeloproliferative disease, including chromosome 10 abnormality, were identified. A normal eosinophil count and disappearance of the chromosomal abnormality were obtained with interferon, hydroxyurea and prednisone therapy. At 30 months postoperatively, the patient had a normal lifestyle.

Severe mitral valve involvement in a child with hypereosinophilia secondary to parasitic infection.

An 11-year-old boy with severe mitral regurgitation due to hypereosinophilia caused by infection with a filaria (Mansonella perstans) required mitral valve replacement with a prosthetic valve. During recurrent postoperative hypereosinophilia, the patient experienced severe mitral stenosis due to thrombosis of the mitral prosthesis. Despite adequate anticoagulation, the prosthesis had to be replaced as an emergency with a second prosthetic valve. Permanent control of the eosinophil count was achieved with chronic oral steroid administration. In contrast to other microfilariae, M. perstans is non-pathogenic to humans; nevertheless, longstanding hypereosinophilia may lead to severe cardiac involvement endangering the patient's life.

Hypereosinophilic sclerosing cholangitis: findings using half-Fourier magnetic resonance imaging.

Hypereosinophilic sclerosing cholangitis is a rare disease caused by eosinophilic infiltration of the gallbladder and biliary tract seen in the idiopathic hypereosinophilic syndrome. We report a 42-year-old woman who presented with symptoms of cholecystitis and obstructive cholangitis. Imaging with magnetic resonance cholangiography using a half-Fourier spinecho sequence, we were able to visualize rapidly and non-invasively a severely abnormal gallbladder, evidence of liver parenchymal inflammation, and biliary duct dilatation.

[A case of surgical treatment for Löffler's endomyocarditis].

We performed surgical treatment in a case of löffler's endocarditis. The patient was a 32-year-old male whose first symptom was easy fatigability. Blood count showed eosinophilia (eosinocyte count 6720/mm3). Echocardiography and vetriculography showed thickened bilateral endocardium and extension disturbance. We diagnosed this case as löffler's endocarditis and performed surgical treatment because medical treatment was unsuccessful. Removal of the thrombus the bilateral ventricles, endocardectomy and mitral valve replacement were performed. Endocardectomy required close attention because the border between thickened endocardium and normal myocardium was obscure. The patient survived surgery, but postoperative echocardiography (15 days) revealed slightly thickened endocardium of the right ventricle. He died of left heart failure 1 month after surgery. At that time, eosinocyte count was 110,000/mm3.

Eosinophilic endocarditis and Strongyloides stercoralis.

A 40-year-old woman from El Salvador presented with 3 months of abdominal pain and diarrhea followed by 2 weeks of atypical chest pain and exertional dyspnea and was diagnosed with eosinophilic endocarditis secondary to Strongyloides stercoralis infection. Transthoracic echocardiogram revealed apical masses in the left and right ventricles and a thickened posterior mitral valve leaflet and cardiac magnetic resonance imaging confirmed the presence of a left ventricular apical mass with diffuse subendocardial delayed enhancement consistent with endocardial fibrosis. In conclusion, eosinophilic endocarditis is a rare cause of restrictive cardiomyopathy characterized by endomyocardial fibrosis and apical thrombosis and fibrosis with frequent involvement of the posterior mitral valve leaflet.

Clinical management of the hypereosinophilic syndromes.

Hypereosinophilic syndromes (HESs) are rare disorders characterized by marked hypereosinophilia that is directly responsible for organ damage or dysfunction. Different pathogenic mechanisms have been discovered in patient subgroups leading to the characterization of myeloproliferative and lymphocytic disease variants. In the updated terminology, idiopathic HES is now restricted to patients with HES of undetermined etiology. The practical clinical approach of patients with the different HES variants is reviewed herein, focusing on specific diagnostic tools and therapeutic options. Corticosteroids, hydroxyurea and IFN-α remain the classical agents for treatment of most patients with HESs. The specific role of therapeutic compounds that have become available more recently, namely, tyrosine kinase inhibitors and IL-5 antagonists, is discussed.

Therapeutic options for patients with clonal and idiopathic hypereosinophia.

BACKGROUND: The hypereosinophilic syndrome (HES) comprises a heterogeneous group of disorders characterized by chronic, unexplained hypereosinophilia with organ involvement. The discovery of novel molecular targets has changed the therapeutic paradigm in HES. OBJECTIVE: This article reviews the current medical management of patients with clonal and idiopathic hypereosinophilia with a particular emphasis on emerging new targeted therapies. METHODS: The information contained in this review was obtained from public sources such as journals and scientific meeting abstracts. The opinions expressed in this review are solely those of the authors. RESULTS/CONCLUSION: The development of imatinib-resistant mutations in the FIP1L1-PDGFR-alpha kinase domain has spurred the development of an array of new tyrosine kinase inhibitors. Moreover, the elucidation of the role of interleukin-5 in the pathogenesis of the lymphocytic variant of HES and the fact that CD52 is expressed on the surface of eosinophils and T cells have led to the clinical use of monoclonal antibodies such as mepolizumab, reslizumumab, and alemtuzumab for the treatment of different forms of hypereosinophilia.

Hypereosinophilic syndrome and myocardial fibrosis.

A 62-year-old man was accepted to our institution because of hypereosinophilia, severe tricuspid regurgitation and isolated right restrictive myocardiopathy, with thrombi inside the right atrium and ventricle. Based on the diagnosis of hypereosinophilic syndrome plus eosinophilic myocarditis, the patient underwent a tricuspid valve repair and endomyocardiectomy. We briefly discuss hypereosinophilic syndrome myocardiopathy, and its management.

[Surgical treatment of the hypereosinophilic syndrome with cardiac involvement (Löffler's endocarditis)]. / Operative Therapie bei Hypereosinophiliesyndrom mit kardialer Beteiligung (Löffler-Endokarditis).

UNLABELLED: HISTORY AND LABORATORY FINDINGS: A 38-year-old woman presented for further treatment of heart failure with NYHA class III symptoms and the idiopathic eosinophilia syndrome. Differential blood count had previously revealed an eosinophilia of 19%, which had fallen to normal under immunosuppression . INVESTIGATIONS: Echocardiography demonstrated echo-rich material in the left ventricular (LV) apex, severe mitral regurgitation and a restriction pattern in the transmitral flow profile. Magnetic resonance imaging (MRI) additionally showed a parietal thrombus sitting on the echo-rich mass in the LV apex. TREATMENT AND COURSE: Surgical LV endocardial resection and allogenic mitral valve replacement were performed. At follow-up (currently four years after the operation) the patient reported marked improvement of symptoms. Echocardiography and MRI demonstrated a normal configuration of the LV apex. CONCLUSION: While endomyocardial fibrosis usually has a poor prognosis, this case illustrates hat surgical treatment of an associated mitral regurgitation can in selected cases be successful.