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• W2022963104 abstract "Idiopathic hypereosinophilic 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 cardiomyopathy. 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. Idiopathic hypereosinophilic 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 cardiomyopathy. 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. Idiopathic hypereosinophilic syndrome (HES) with endomyocardial disease has been well described in the literature.1Chusid MJ Dale DC West BC Wolff SM The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature.Medicine (Baltimore). 1975; 54: 1-27Crossref PubMed Scopus (1196) Google Scholar, 2Parrillo JE Borer JS Henry WL Wolff SM Fauci AS The cardiovascular manifestations of the hypereosinophilic syndrome: prospective study of 26 patients, with review of the literature.Am J Med. 1979; 67: 572-582Abstract Full Text PDF PubMed Scopus (258) Google Scholar, 3Schooley RT Flaum MA Gralnick HR Fauci AS A clinicopathologic correlation of the idiopathic hypereosinophilic syndrome, II: clinical manifestations.Blood. 1981; 58: 1021-1026PubMed Google Scholar, 4Fauci AS Harley JB Roberts WC Ferrans VJ Gralnick HR Bjornson BH The idiopathic hypereosinophilic syndrome: clinical, pathophysiologic, and therapeutic considerations.Ann Intern Med. 1982; 97: 78-92Crossref PubMed Google Scholar, 5Spry CJ The hypereosinophilic syndrome: clinical features, laboratory findings and treatment.Allergy. 1982; 37: 539-551Crossref PubMed Scopus (106) Google Scholar However, the diagnosis of this entity can be difficult because presentation of the patients and progression of the disease are variable. Historically, most patients have had peripheral eosinophilia for a long time. We describe a patient with no evidence of hypereosinophilia in whom cardiac biopsy revealed eosinophilic infiltration of the endomyocardium. This atypical presentation illustrates the challenge in diagnosing and treating this rare form of restrictive cardiomyopathy. A 74-year-old man with a history of coronary heart disease but no known myocardial infarction had been in apparently good health until 3 weeks previously, when increasing dyspnea and fatigue developed. He presented to his local physician. After transesophageal echocardiography showed a large left ventricular (LV) apical thrombus, the patient was admitted to a local hospital. Laboratory studies revealed an eosinophil count of 1.11 × 109Felice PV Sawicki J Anto J Endomyocardial disease and eosinophilia.Angiology. 1993; 44: 869-874Crossref PubMed Scopus (23) Google Scholar/L, and bone marrow biopsy tissue was negative for hematologic disease. The patient was transferred to our institution for evaluation of the LV mass. On admission, the patient denied chest pain, palpitations, syncope, ankle edema, nausea, and vomiting. He stated that a raspy voice and cough had developed in recent weeks and that he had had pruritus of the lower back and lower extremities during the previous 6 months. He had traveled to Mexico annually for the past several years. The patient's medical history was notable for coronary artery disease, hypertension, hyperlipidemia, type 2 diabetes mellitus, chronic renal insufficiency, gout, and anemia. Current medications included aspirin, allopurinol, atenolol, fluvastatin, metformin, and triamterene-hydrochlorothiazide. His family history was remarkable for ischemic heart disease. On physical examination, the patient appeared fatigued but not acutely ill. His temperature was 36.9°C, blood pressure was 110/48 mm Hg, pulse rate was 69/min, and respirations were 16/min. On cardiovascular examination, the heart rate and rhythm were regular, S1 and S2 were normal, and a 2/6 ejection murmur was heard, best at the right upper sternal border. Jugular venous pressure was 10 cm H2O. Carotid artery pulses were brisk and equal bilaterally. No carotid, aortic, or femoral bruits were heard. Peripheral pulses were intact and symmetrical. No peripheral edema, clubbing, or cyanosis was present. Pulmonary and abdominal examination findings were essentially normal. The patient had multiple excoriations over the abdomen, back, and lower extremities. Laboratory test results were as follows: hemoglobin, 11.7 g/dL; hematocrit, 35.3%; leukocytes, 7.0 × 109Felice PV Sawicki J Anto J Endomyocardial disease and eosinophilia.Angiology. 1993; 44: 869-874Crossref PubMed Scopus (23) Google Scholar/L; eosinophils, 0.50 × 109Felice PV Sawicki J Anto J Endomyocardial disease and eosinophilia.Angiology. 1993; 44: 869-874Crossref PubMed Scopus (23) Google Scholar/L; and platelets, 174 × 109Felice PV Sawicki J Anto J Endomyocardial disease and eosinophilia.Angiology. 1993; 44: 869-874Crossref PubMed Scopus (23) Google Scholar/L. Chemistry panel results were notable only for a creatinine level of 1.7 mg/dL. The erythrocyte sedimentation rate (55 mm/h), high-sensitivity C-reactive protein level (2.58 mg/L), and antinuclear antibody level (5.7) were elevated. The cytoplasmic antineutrophil cytoplasmic autoantibody titer was negative, but the perinuclear antineutrophil cytoplasmic autoantibody titer was positive. Concentrations of myeloperoxidase antibodies and C3, C4, and total complement were within the reference ranges. Results of special coagulation studies were negative. Urinalysis showed 1% to 5% eosinophils. Chest radiography revealed left pleural effusion and mild cardiomegaly. Pleural fluid from thoracentesis was negative for malignancy. Bone marrow biopsy tissue collected at the patient's local hospital was reviewed by our pathologists and interpreted as normocellular with normal trilineage hematopoiesis. Normochromic normocytic anemia was identified on the peripheral blood smear. Electrocardiography showed a prolonged QT interval and sinus bradycardia with ST-segment and T-wave abnormalities suggestive of inferior and anterolateral ischemia. Transesophageal echocardiography revealed a gelatinous 2.9-cm mass with no mobile components occupying the entire LV apex. The LV ejection fraction was reduced to 45% because of apical akinesis. A restrictive filling pattern (grade 3/4) was evident, with markedly elevated LV filling pressures. The aortic and mitral valves were mildly regurgitant, and the left atrium and left ventricle were slightly enlarged. Right ventricular (RV) size and function were normal (Figure 1). Magnetic resonance imaging (MRI) of the heart showed a suspected mass in the LV apex on black-blood sequences (Figure 2, left). The suspected mass appeared to be a thickening of akinetic apical myocardium on white-blood cine sequences (Figure 2, right). Gated computed tomography (CT) of the heart clearly identified a noncalcified mass filling much of the LV apex. Most of the mass showed no enhancement, although a rim of hyperenhancement was seen on delayed imaging (Figure 3, left). The mass abutted and adhered to the myocardium but did not appear to invade it. No LV aneurysm or diverticulum was noted (Figure 3, right). The RV apex appeared normal, with no evidence of thrombus. Lung lesions and hilar lymphadenopathy were not apparent. Coronary angiography confirmed the presence of the LV mass and severe atherosclerotic coronary artery disease, with 50% obstruction in the left main coronary artery and 70% to 90% obstruction in the left anterior descending coronary artery and in the circumflex artery, its first 2 obtuse marginal branches, and its posterior descending branch. Right ventricular endomyocardial biopsy specimens showed mild focal perivascular inflammatory infiltrates (primarily lymphocytic with infrequent eosinophils and macrophages) and mild focal replacement fibrosis, which according to the Dallas criteria represents borderline myocarditis. The perivascular distribution suggested a drug-related phenomenon. No granuloma, mural thrombus, amyloid, or stainable iron was identified. The patient underwent bypass grafting of 3 coronary arteries and resection of the LV thrombus (Figure 4). Microscopic evaluation of the resected LV tissue revealed a large, old, degenerating fibrin-rich mural thrombus with infrequent eosinophils. The base of the thrombus was organized, producing bandlike endocardial granulation tissue and dense fibrosis. Microfocal noncaseating granulomatous inflammation was multifocal, involving subjacent myocardium and perivascular and interstitial tissue. The microgranulomas were suggestive of hypersensitivity myocarditis or possibly sarcoidosis. However, approximately 75% of the resected thrombotic tissue was positive for eosinophilic granule major basic protein on immuno-fluorescence staining (Figure 5).FIGURE 5Immunofluorescence photomicrographs of left ventricular mural thrombus showing eosinophilic granule major basic protein. Left, Section stained with affinity-purified rabbit anti–human major basic protein shows extensive diffuse deposition of cytotoxic major basic protein from degranulated eosinophils (original magnification ×160). Right, Old degenerating thrombus with rare intact eosinophils (hematoxylin-eosin, original magnification ×150).View Large Image Figure ViewerDownload (PPT) The patient's postoperative course was uneventful, and he was dismissed from the hospital 8 days postoperatively. Oral anticoagulation was initiated. When the patient was seen at follow-up 3 months later, he had completed his cardiac rehabilitation program and remained asymptomatic. Follow-up transthoracic echocardiography at that time showed an LV ejection fraction of 55% and persistent akinesis of the apex but no recurrence of thrombus in the apex. The differential diagnosis in our patient included causes of ventricular mural thrombus and peripheral blood eosinophilia. In addition, biventricular tissue inflammation suggested drug-related myocarditis or a hypersensitivity reaction. Given the patient's history of ischemic heart disease and the echocardiographic features of the apical akinesis, stasis thrombosis was a likely possibility. Laboratory reports from his local medical institution indicated a slight peripheral blood eosinophilia, although serial eosinophil counts obtained at our institution were consistently at the upper limit of the reference range. When immunofluorescence staining of the resected LV tissue showed large concentrations of eosinophilic granule major basic protein, a definite diagnosis of eosinophilic endomyocardial disease (EED) was established. Inasmuch as possible causes of both clonal and nonclonal peripheral blood eosinophilia were ruled out, idiopathic HES with endomyocardial disease became the final diagnosis. Although the association between peripheral eosinophilia and target-organ damage was first noted more than 100 years ago, it was not until 1968 that Hardy and Anderson6Hardy WR Anderson RE The hypereosinophilic syndromes.Ann Intern Med. 1968; 68: 1220-1229Crossref PubMed Scopus (408) Google Scholar codified these entities into a spectrum of diseases that they termed hypereosinophilic syndromes. In 1975, Chusid et al1Chusid MJ Dale DC West BC Wolff SM The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature.Medicine (Baltimore). 1975; 54: 1-27Crossref PubMed Scopus (1196) Google Scholar reviewed the findings in patients with eosinophilia referred to the National Institutes of Health and those in other HES patients and presented the 3 defining features of HES, which are still considered valid today (Table 1). In addition to these criteria, total leukocyte counts are usually elevated, with 30% to 70% eosinophils, and absolute neutrophilia may be present. Bone marrow eosinophilia ranges from 30% to 60%, often with a left shift in maturation.7Flaum MA Schooley RT Fauci AS Gralnick HR A clinicopathologic correlation of the idiopathic hypereosinophilic syndrome, I: hematologic manifestations.Blood. 1981; 58: 1012-1020PubMed Google ScholarTABLE 1Diagnostic Criteria for Idiopathic Hypereosinophilic Syndrome Eosinophil count >1.5 × 109/L for ≥6 mo (upper limit of reference range, 0.50 × 109/L)No evidence of parasitic, allergic, or other known causes of eosinophiliaSigns or symptoms of organ involvement that appear related to eosinophilia (heart, nervous system, skin, lungs, liver, gastrointestinal tract)Data from Chusid et al.1Chusid MJ Dale DC West BC Wolff SM The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature.Medicine (Baltimore). 1975; 54: 1-27Crossref PubMed Scopus (1196) Google Scholar Open table in a new tab Data from Chusid et al.1Chusid MJ Dale DC West BC Wolff SM The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature.Medicine (Baltimore). 1975; 54: 1-27Crossref PubMed Scopus (1196) Google Scholar The prevalence of HES is unknown, although Spry5Spry CJ The hypereosinophilic syndrome: clinical features, laboratory findings and treatment.Allergy. 1982; 37: 539-551Crossref PubMed Scopus (106) Google Scholar postulated a rate of 1 case per 200,000 people. The disease is more common in men and tends to occur between the ages of 20 and 50 years.1Chusid MJ Dale DC West BC Wolff SM The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature.Medicine (Baltimore). 1975; 54: 1-27Crossref PubMed Scopus (1196) Google Scholar Various organs may be involved, but those most commonly affected are the heart, brain, skin, and lungs.1Chusid MJ Dale DC West BC Wolff SM The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature.Medicine (Baltimore). 1975; 54: 1-27Crossref PubMed Scopus (1196) Google Scholar, 2Parrillo JE Borer JS Henry WL Wolff SM Fauci AS The cardiovascular manifestations of the hypereosinophilic syndrome: prospective study of 26 patients, with review of the literature.Am J Med. 1979; 67: 572-582Abstract Full Text PDF PubMed Scopus (258) Google Scholar, 5Spry CJ The hypereosinophilic syndrome: clinical features, laboratory findings and treatment.Allergy. 1982; 37: 539-551Crossref PubMed Scopus (106) Google Scholar, 8Karnak D Kayacan O Beder S Delibalta M Hypereosinophilic syndrome with pulmonary and cardiac involvement in a patient with asthma.CMAJ. 2003; 168: 172-175PubMed Google Scholar The primary cause of morbidity and death is cardiac involvement, which occurs in more than 75% of patients with idiopathic HES.9Felice PV Sawicki J Anto J Endomyocardial disease and eosinophilia.Angiology. 1993; 44: 869-874Crossref PubMed Scopus (23) Google Scholar, 10Solley GO Maldonado JE Gleich GJ et al.Endomyocardiopathy with eosinophilia.Mayo Clin Proc. 1976; 51: 697-708PubMed Google Scholar The mechanism of tissue damage has not been delineated, although the cytotoxic effects of proteins produced by eosinophils are probably important.11Tai PC Ackerman SJ Spry CJ Dunnette S Olsen EG Gleich GJ Deposits of eosinophil granule proteins in cardiac tissues of patients with eosinophilic endomyocardial disease.Lancet. 1987; 1: 643-647Abstract PubMed Scopus (244) Google Scholar, 12deMello DE Liapis H Jureidini S Nouri S Kephart GM Gleich GJ Cardiac localization of eosinophil-granule major basic protein in acute necrotizing myocarditis.N Engl J Med. 1990; 323: 1542-1545Crossref PubMed Scopus (97) Google Scholar In peripheral blood, eosinophils have a half-life of approximately 8 to 18 hours. However, after they are recruited in peripheral tissues by specific cytokines, eosinophils can survive for days or weeks as resident cells.13Lombardi C Passalacqua G Eosinophilia and diseases: clinical revision of 1,862 cases.Arch Intern Med. 2003; 163: 1371-1373Crossref PubMed Google Scholar In patients with HES, circulating eosinophils generally have measurable structural and functional abnormalities, and many of them are degranulated.10Solley GO Maldonado JE Gleich GJ et al.Endomyocardiopathy with eosinophilia.Mayo Clin Proc. 1976; 51: 697-708PubMed Google Scholar Sequestration of eosinophils in the endocardium and in other organ tissues or systems occurs by unknown mechanisms.14Leiferman KM Gleich GJ Hypereosinophilic syndrome: case presentation and update.J Allergy Clin Immunol. 2004; 113: 50-58Abstract Full Text Full Text PDF PubMed Scopus (49) Google Scholar Eosinophil-derived neurotoxin, eosinophil cationic protein, and major basic protein are enzymes released by eosinophils that cause endothelial damage and promote thrombosis.11Tai PC Ackerman SJ Spry CJ Dunnette S Olsen EG Gleich GJ Deposits of eosinophil granule proteins in cardiac tissues of patients with eosinophilic endomyocardial disease.Lancet. 1987; 1: 643-647Abstract PubMed Scopus (244) Google Scholar, 12deMello DE Liapis H Jureidini S Nouri S Kephart GM Gleich GJ Cardiac localization of eosinophil-granule major basic protein in acute necrotizing myocarditis.N Engl J Med. 1990; 323: 1542-1545Crossref PubMed Scopus (97) Google Scholar, 15Weller PF Bubley GJ The idiopathic hypereosinophilic syndrome.Blood. 1994; 83: 2759-2779PubMed Google Scholar Intact eosinophils and extracellular granules are present in affected tissues throughout most of the disease process; however, only granules remain during the final fibrotic stage of the disease. The correlation between the extent of tissue involvement and the degree or duration of peripheral blood eosinophilia has been debated.1Chusid MJ Dale DC West BC Wolff SM The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature.Medicine (Baltimore). 1975; 54: 1-27Crossref PubMed Scopus (1196) Google Scholar, 13Lombardi C Passalacqua G Eosinophilia and diseases: clinical revision of 1,862 cases.Arch Intern Med. 2003; 163: 1371-1373Crossref PubMed Google Scholar, 15Weller PF Bubley GJ The idiopathic hypereosinophilic syndrome.Blood. 1994; 83: 2759-2779PubMed Google Scholar, 16Parrillo JE Fauci AS Wolff SM Therapy of the hypereosinophilic syndrome.Ann Intern Med. 1978; 89: 167-172Crossref PubMed Scopus (233) Google Scholar, 17Kobayashi S Inokuma S Setoguchi K Kono H Abe K Incidence of peripheral blood eosinophilia and the threshold eosinophile count for indicating hypereosinophilia-associated diseases.Allergy. 2002; 57: 950-956Crossref PubMed Scopus (22) Google Scholar Kobayashi et al17Kobayashi S Inokuma S Setoguchi K Kono H Abe K Incidence of peripheral blood eosinophilia and the threshold eosinophile count for indicating hypereosinophilia-associated diseases.Allergy. 2002; 57: 950-956Crossref PubMed Scopus (22) Google Scholar and Leiferman and Gleich14Leiferman KM Gleich GJ Hypereosinophilic syndrome: case presentation and update.J Allergy Clin Immunol. 2004; 113: 50-58Abstract Full Text Full Text PDF PubMed Scopus (49) Google Scholar suggested that the threshold eosinophil count for the diagnosis and treatment of hypereosinophilic disease is 2 × 109Felice PV Sawicki J Anto J Endomyocardial disease and eosinophilia.Angiology. 1993; 44: 869-874Crossref PubMed Scopus (23) Google Scholar/L or greater for a prolonged period because that level of eosinophilia seems to be associated with the greatest risk of organ involvement. However, Fauci et al4Fauci AS Harley JB Roberts WC Ferrans VJ Gralnick HR Bjornson BH The idiopathic hypereosinophilic syndrome: clinical, pathophysiologic, and therapeutic considerations.Ann Intern Med. 1982; 97: 78-92Crossref PubMed Google Scholar reported that peripheral eosinophil counts may remain normal for weeks or months during the course of the disease, and Schooley et al3Schooley RT Flaum MA Gralnick HR Fauci AS A clinicopathologic correlation of the idiopathic hypereosinophilic syndrome, II: clinical manifestations.Blood. 1981; 58: 1021-1026PubMed Google Scholar suggested that factors other than the absolute number of circulating eosinophils may contribute to the severity of the disease (eg, maturity, granule content, and migration patterns). Our patient had no documented history of sustained peripheral blood eosinophilia over a long period before his evaluation at our institution, although he had not been followed up regularly by a physician and thus had minimal prior laboratory study results available in his medical record. His eosinophil count was slightly elevated on presentation at our institution but subsequently normalized without treatment. This presented a diagnostic dilemma because our patient did not meet the classic criteria for a diagnosis of idiopathic HES with cardiac involvement. Chusid et al1Chusid MJ Dale DC West BC Wolff SM The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature.Medicine (Baltimore). 1975; 54: 1-27Crossref PubMed Scopus (1196) Google Scholar were the first to describe 3 stages of EED. With chronic eosinophilia, eosinophilic myocarditis develops because eosinophilic infiltration of the endocardium and myocardium leads to microabscesses. This first stage is the necrotic stage. The second, or thrombotic, stage is characterized by the formation of thrombi along the damaged endocardium of either or both ventricles and occasionally the atria. Typically, the ventricular outflow tracts and semilunar valves are spared. Thrombi may also form on the atrioventricular valve leaflets. The third stage is fibrotic. Morphologic findings at this stage include mural thrombus and fibrotic thickening of the endocardium, which affects the apex and inflow tract of one or both ventricles and frequently involves one or both atrioventricular valves.4Fauci AS Harley JB Roberts WC Ferrans VJ Gralnick HR Bjornson BH The idiopathic hypereosinophilic syndrome: clinical, pathophysiologic, and therapeutic considerations.Ann Intern Med. 1982; 97: 78-92Crossref PubMed Google Scholar Entrapment of leaflets and chordae tendineae cordis may cause mitral or tricuspid regurgitation or both. Common clinical manifestations of EED include cardiomegaly, congestive heart failure (right-sided, left-sided, or both), atrioventricular valve regurgitation, arrhythmias, and restrictive cardiomyopathy.1Chusid MJ Dale DC West BC Wolff SM The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature.Medicine (Baltimore). 1975; 54: 1-27Crossref PubMed Scopus (1196) Google Scholar, 2Parrillo JE Borer JS Henry WL Wolff SM Fauci AS The cardiovascular manifestations of the hypereosinophilic syndrome: prospective study of 26 patients, with review of the literature.Am J Med. 1979; 67: 572-582Abstract Full Text PDF PubMed Scopus (258) Google Scholar, 5Spry CJ The hypereosinophilic syndrome: clinical features, laboratory findings and treatment.Allergy. 1982; 37: 539-551Crossref PubMed Scopus (106) Google Scholar, 18D'Souza MG Swistel DG Castro JL DeRose Jr, JJ Hypereosinophilic thrombus causing aortic stenosis and myocardial infarction.Ann Thorac Surg. 2003; 76: 1725-1726Abstract Full Text Full Text PDF PubMed Scopus (8) Google Scholar Typically, diagnostic tests yield a constellation of findings that suggest EED. Chest radiographs frequently show cardiomegaly and pulmonary congestion or less often pulmonary infiltrates. The most common electrocardiographic abnormalities are nonspecific ST-segment and T-wave changes (which our patient had), ventricular premature beats, and LV hypertrophy.2Parrillo JE Borer JS Henry WL Wolff SM Fauci AS The cardiovascular manifestations of the hypereosinophilic syndrome: prospective study of 26 patients, with review of the literature.Am J Med. 1979; 67: 572-582Abstract Full Text PDF PubMed Scopus (258) Google Scholar Other reported disturbances include marked ST-segment depression,19Maruyoshi H Nakatani S Yasumura Y et al.Löffler's endocarditis associated with unusual ECG change mimicking posterior myocardial infarction.Heart Vessels. 2003; 18: 43-46Crossref PubMed Scopus (11) Google Scholar low-voltage QRS complex,20Kabbani SS LeWinter MM Diastolic heart failure: constrictive, restrictive, and pericardial.Cardiol Clin. 2000; 18: 501-509Abstract Full Text Full Text PDF PubMed Scopus (15) Google Scholar arrhythmias (especially atrial fibrillation),21Arnold M McGuire L Lee JC Loeffler's fibroplastic endocarditis.Pathology. 1988; 20: 79-82Crossref PubMed Scopus (6) Google Scholar and conduction disturbances, particularly right bundle branch block when the RV apex is fibrotic.1Chusid MJ Dale DC West BC Wolff SM The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature.Medicine (Baltimore). 1975; 54: 1-27Crossref PubMed Scopus (1196) Google Scholar Echocardiographic assessment is key to the diagnosis of EED. The most common findings are ventricular apical obliteration and posterior mitral leaflet thickening with absent or markedly limited motion.2Parrillo JE Borer JS Henry WL Wolff SM Fauci AS The cardiovascular manifestations of the hypereosinophilic syndrome: prospective study of 26 patients, with review of the literature.Am J Med. 1979; 67: 572-582Abstract Full Text PDF PubMed Scopus (258) Google Scholar, 22Ommen SR Seward JB Tajik AJ Clinical and echocardiographic features of hypereosinophilic syndromes.Am J Cardiol. 2000; 86: 110-113Abstract Full Text Full Text PDF PubMed Scopus (128) Google Scholar Additional findings may include atrial enlargement and evidence of atrioventricular valve regurgitation. Systolic function is often well preserved, but Doppler flow imaging reveals a physiologically restrictive pattern, which is most likely due to endomyocardial fibrosis. However, findings of a recent strain and strain-rate study suggested that the restrictive pattern may result solely from a decrease in end-systolic cavity size due to the presence of the mural thrombus.23Eroglu E Di Salvo G Herbots L Herregods MC Sutherland GR Restrictive left ventricular filling in hypereosinophilic syndrome as a result of partial cavity obliteration by an apical mass: a strain/strain rate study.J Am Soc Echocardiogr. 2003; 16: 1088-1090Abstract Full Text Full Text PDF PubMed Scopus (10) Google Scholar Our patient had echocardiographic evidence consistent with these findings, including the presence of an apical LV mass and aortic and mitral valve regurgitation. Normally, cardiac catheterization shows elevated RV and LV end-diastolic pressures, and cardiac angiography shows apical ventricular obliteration and valvular incompetence.9Felice PV Sawicki J Anto J Endomyocardial disease and eosinophilia.Angiology. 1993; 44: 869-874Crossref PubMed Scopus (23) Google Scholar, 19Maruyoshi H Nakatani S Yasumura Y et al.Löffler's endocarditis associated with unusual ECG change mimicking posterior myocardial infarction.Heart Vessels. 2003; 18: 43-46Crossref PubMed Scopus (11) Google Scholar Magnetic resonance imaging of the heart is being used increasingly to assist in diagnosing EED because of its ability to demonstrate the presence and extent of infiltration and to localize the site for endomyocardial biopsy. Magnetic resonance imaging findings suggestive of EED include a marked signal increase in the apical portion of the interventricular septum and apex, predominantly in the subendocardium, which suggests the presence of infiltration.13Lombardi C Passalacqua G Eosinophilia and diseases: clinical revision of 1,862 cases.Arch Intern Med. 2003; 163: 1371-1373Crossref PubMed Google Scholar Cine MRI typically shows akinesia of the apical LV wall and thrombus in the LV apex, RV apex, or both.24Puvaneswary M Joshua F Ratnarajah S Idiopathic hypereosinophilic syndrome: magnetic resonance imaging findings in endomyocardial fibrosis.Australas Radiol. 2001; 45: 524-527Crossref PubMed Scopus (25) Google Scholar The MRI that was obtained in our case was inadequate to establish a definitive diagnosis. The addition of a perfusion sequence and postcontrast sequences to detect myocardial or any mass enhancement likely would have provided the necessary information, and CT would not have been performed.25Mollet NR Dymarkowski S Volders W et al.Visualization of ventricular thrombi with contrast-enhanced magnetic resonance imaging in patients with ischemic heart disease.Circulation. 2002; 106: 2873-2876Crossref PubMed Scopus (235) Google Scholar, 26Bishop GG Bergin JD Kramer CM Hypereosinophilic syndrome and restrictive cardiomyopathy due to apical thrombi.Circulation. 2001; 104: E3-E4Crossref PubMed Google Scholar, 27Kim RJ Fieno DS Parrish TB et al.Relationship of MRI delayed contrast enhancement to irreversible injury, infarct age, and contractile function.Circulation. 1999; 100: 1992-2002Crossref PubMed Scopus (2097) Google Scholar In view of the MRI results, contrast CT was performed to define more clearly the character of the mass. Endomyocardial biopsy is the gold standard for diagnosing EED, although the biopsy findings are not always positive. Because the disease is often focal, RV biopsy sampling may miss left-sided disease, as in our patient. A left-sided biopsy specimen is not recommended because sampling may dislodge the mural thrombus, with resultant systemic embolization. Traditionally, 3 criteria are used to diagnose EED: (1) the criteria for HES must be met, (2) there must be pathologic evidence of eosinophil infiltration with degranulation, and (3) classic cardiac features must be evident. Our patient did not have peripheral eosinophilia while at our institution, so he did not meet the classic criteria for HES. However, other cases of possible HES with EED but no peripheral blood eosinophilia at the time of diagnosis have been reported.28Priglinger U Drach J Ullrich R Baumgartner H Huber K Maurer G Idiopathic eosinophilic endomyocarditis in the absence of peripheral eosinophilia.Leuk Lymphoma. 2002; 43: 215-218Crossref PubMed Scopus (10) Google Scholar, 29De Vriese AS Kips JC Vogelaers DP Vandewoude KH Cuvelier CA Colardyn FA Pitfalls in the diagnosis of hypereosinophilic syndrome: a report of two cases.J Intern Med. 1997; 241: 165-170Crossref PubMed Scopus (23) Google Scholar The reasons for this discrepancy are unclear, but possible mechanisms include dysregulation in eosinophil dynamics or a defect in eosinophil migration.28Priglinger U Drach J Ullrich R Baumgartner H Huber K Maurer G Idiopathic eosinophilic endomyocarditis in the absence of peripheral eosinophilia.Leuk Lymphoma. 2002; 43: 215-218Crossref PubMed Scopus (10) Google Scholar Possibly, cases with an atypical presentation may be underreported in the literature. Treatment of EED focuses on long-term maintenance to control eosinophilia and prevent further damage to the cardiovascular or other organ systems. Supportive treatment for congestive heart failure, including digitalis, diuretics, and afterload reduction, is often necessary. The use of anticoagulation in HES patients is debatable—some investigators do not recommend its use because it seems ineffective in preventing further thrombosis,5Spry CJ The hypereosinophilic syndrome: clinical features, laboratory findings and treatment.Allergy. 1982; 37: 539-551Crossref PubMed Scopus (106) Google Scholar but others do advocate its use in HES patients presenting with a thrombotic process.15Weller PF Bubley GJ The idiopathic hypereosinophilic syndrome.Blood. 1994; 83: 2759-2779PubMed Google Scholar Serial eosinophil counts are recommended. Regularly scheduled cardiac function testing is mandatory because, with the possibility of embolization, a new endocardial lesion warrants immediate medical, and occasionally surgical, attention. The National Institutes of Health produced a scoring system to predict which patients with HES need intensive medical therapy.3Schooley RT Flaum MA Gralnick HR Fauci AS A clinicopathologic correlation of the idiopathic hypereosinophilic syndrome, II: clinical manifestations.Blood. 1981; 58: 1021-1026PubMed Google Scholar Various treatment algorithms have been proposed,15Weller PF Bubley GJ The idiopathic hypereosinophilic syndrome.Blood. 1994; 83: 2759-2779PubMed Google Scholar, 16Parrillo JE Fauci AS Wolff SM Therapy of the hypereosinophilic syndrome.Ann Intern Med. 1978; 89: 167-172Crossref PubMed Scopus (233) Google Scholar with glucocorticoids generally accepted as being first-line therapy. Adjunctive or alternative therapies include hydroxyurea, vincristine, alkylating agents, and etoposide. Interferon alfa30Butterfield JH Gleich GJ Interferon-alpha treatment of six patients with the idiopathic hypereosinophilic syndrome.Ann Intern Med. 1994; 121: 648-653Crossref PubMed Scopus (115) Google Scholar, 31Baratta L Afeltra A Delfino M De Castro S Giorgino F Rossi-Fanelli F Favorable response to high-dose interferon-alpha in idiopathic hypereosinophilic syndrome with restrictive cardiomyopathy: case report and literature review.Angiology. 2002; 53: 465-470Crossref PubMed Scopus (25) Google Scholar and imatinib mesylate32Cortes J Ault P Koller C et al.Efficacy of imatinib mesylate in the treatment of idiopathic hypereosinophilic syndrome.Blood. 2003; 101: 4714-4716Crossref PubMed Scopus (149) Google Scholar, 33Gleich GJ Leiferman KM Pardanani A Tefferi A Butterfield JH Treatment of hypereosinophilic syndrome with imatinib mesilate.Lancet. 2002; 359: 1577-1578Abstract Full Text Full Text PDF PubMed Scopus (303) Google Scholar have shown promising results in selected patient populations. Currently, anti–interleukin 5 is under investigation as a possible corticosteroid-sparing agent.34Garrett JK Jameson SC Thomson B et al.Anti-interleukin-5 (mepolizumab) therapy for hypereosinophilic syndromes.J Allergy Clin Immunol. 2004; 113: 115-119Abstract Full Text Full Text PDF PubMed Scopus (365) Google Scholar If the disease is extremely aggressive, allogeneic bone marrow transplantation may be performed.35Bain BJ Hypereosinophilia.Curr Opin Hematol. 2000; 7: 21-25Crossref PubMed Scopus (63) Google Scholar After the fibrotic stage of EED has occurred, surgical resection may offer clinically meaningful palliation of symptoms. Typically, decortication (endocardiectomy), with or without valve replacement, is performed. Surgery is not without risk, however: the mortality rate is 16% to 20% for univentricular disease and 40% for biventricular disease.36Moraes CR Buffolo E Lima R et al.Surgical treatment of endomyocardial fibrosis.J Thorac Cardiovasc Surg. 1983; 85: 738-745PubMed Google Scholar, 37Metras D Coulibaly AO Ouattara K et al.Endomyocardial fibrosis: early and late results of surgery in 20 patients.J Thorac Cardiovasc Surg. 1982; 83: 52-64PubMed Google Scholar, 38Arvay A Lengyel M Meszaros R Palik I Surgical experience with thrombotic and fibrotic forms of non-tropical eosinophilic endomyocardial disease.Thorac Cardiovasc Surg. 1985; 33: 314-316Crossref PubMed Scopus (4) Google Scholar Late postoperative death, primarily related to prosthetic valve disorders, occurs in 12% of patients.38Arvay A Lengyel M Meszaros R Palik I Surgical experience with thrombotic and fibrotic forms of non-tropical eosinophilic endomyocardial disease.Thorac Cardiovasc Surg. 1985; 33: 314-316Crossref PubMed Scopus (4) Google Scholar After resection, endocardial fibrosis usually does not recur. The manifestations of HES with endomyocardial disease are not uniform, and our case illustrates some of the difficulties that may be encountered in diagnosing this entity. Our patient clearly had EED but lacked evidence of notable sustained peripheral eosinophilia at the onset of symptoms. Causes of EED were ruled out systematically, leading to the diagnosis of HES. Similar cases may be underdiagnosed and underreported in the literature. Endomyocardial biopsy is essential, and it should be considered for all patients who have unexplained heart failure or ventricular arrhythmias. Because patients with advanced disease have a poor prognosis, early diagnosis is crucial to initiate appropriate therapy that may slow the progression of the disease." @default.
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