History

Fact Explanation
Is an idiopathic disease Hyper eosinophilc syndrome (HES) is a poorly understood syndrome characterized by the presence of persistent hypereosinophilia (>1,500 eosinophils 106 /L of peripheral blood for >6 months) and signs or symptoms of multisystemic organ dysfunction in the absence of other known causes of eosinophilia. Cardiac involvement in this is present in 50%- 60% and is termed as Loeffler's endocarditis or eosinophilic endomyocardial disease. The biopsy specimens show the presence of degranulated eosinophils or eosinophilc cationic proteins in the endocardium and activated eosinophils in the myocardium. Endocardium gets thickened as well as affecting the myocardium. In this eosinophils may be associated with myocardial inflammation in three distinct forms. The first is a hypersensitivity reaction to a foreign antigen known as allergic esosinophilic myocarditis and is often drug induced. Then a thrombotic stage resulting in splenic, renal, retinal, cerebral thrombosis. Finally it may present in the form of fulminant necrotic myocarditis of unclear etiology. [1] [2] [3] [4] [7] [8]
Exertional chest pain [2] [3] [5] [6] In the early stage of the illness due to necrosis of cardiac myocytes, there could be carditis, resulting in chest pain. [2] [3] [5] [6]
Ankle swelling [2] [3] In later stages due to fibrosis of the endocardium and myocardium, there's restrictive cardiomyopathy resulting in bi ventricular failure, a diastolic failure to be exact, hence ankle swelling. [2] [3] [7]
Shortness of breath [2] [3] In later stages due to fibrosis of the endocardium and myocardium, there's restrictive cardiomyopathy resulting in bi ventricular failure,a diastolic failure to be exact, and left ventricular failure results in pulmonary congestion hence this [2] [3] [7]
Orthopnoea/ Paroxymal nocturnal dyspnoea [2] [3] In later stages due to fibrosis of the endocardium and myocardium, there's restrictive cardiomyopathy resulting in biventricular failure, and left ventricular failure results in pulmonary congestion hence this [2] [3] [7]
weight loss [2] [3] This is a symptom of acute carditis, resulting in release of cytokines from eosinophils and causing constitutional symptoms associated with this [2] [3]
Fever [2] [3] This is a symptom of hypereosinophilia, resulting in release of cytokines from eosinophils and causing constitutional symptoms associated with this [2] [3]
Cough [2] [3] This is a symptom of acute carditis, associated with this [2] [3]
pruritic skin rash [2] [3] This is a symptom of hyper eosinophilia, resulting in release of cytokines from eosinophils and causing constitutional symptoms associated with this [2] [3]
Excessive sweating [2] [3] This is a symptom of hyper eosinophilia resulting in release of cytokines from eosinophils and causing constitutional symptoms associated with this [2] [3]
Palpitations [2] [3] Due to myocarditis, there could be abnormal rhythm generation from the heart causing ventricular arrhythmias. [2] [3]
Hemiparesis/ Aphasia (Transient ischemia like manifestaions) [3] [4] In this, eosinophil granule proteins, especially MBP1 and the eosinophil peroxidase, can activate platelets and impair the anticoagulant effects of thrombomodulin and cause thrombosis and embolism to the brain and transient ischemic syndrome like manifestations [3] [4]
Sudden death [3] [4] Due to ventricular arrhythmias ultimately causing ventricular fibrillation and due to cerebrovascular accidents both can result in sudden death [3] [4]
References
  1. HAJSADEGHI SHOKOUFEH, CHITSAZAN MITRA, POURALIAKBAR HAMID REZA, SADEGHIPOUR ALIREZA. From an isolated right ventricular thrombus to the diagnosis of the hypereosinophilic syndrome. Journal of Cardiology Cases [online] 2011 June, 3(3):e133-e136 [viewed 05 July 2014] Available from: doi:10.1016/j.jccase.2011.03.002
  2. ACQUATELLA H., SCHILLER N. B., PUIGBO J. J., GOMEZ-MANCEBO J. R., SUAREZ C., ACQUATELLA G.. Value of two-dimensional echocardiography in endomyocardial disease with and without eosinophilia. A clinical and pathologic study. Circulation [online] 1983 June, 67(6):1219-1226 [viewed 05 July 2014] Available from: doi:10.1161/01.CIR.67.6.1219
  3. RIZKALLAH J, DESAUTELS A, MALIK A, ZIEROTH S, JASSAL D, HUSSAIN F, CORDOVA F. Eosinophilic myocarditis: two case reports and review of the literature BMC Res Notes [online] :538 [viewed 06 July 2014] Available from: doi:10.1186/1756-0500-6-538
  4. WRIGHT BENJAMIN L., LEIFERMAN KRISTIN M., GLEICH GERALD J.. Eosinophil Granule Protein Localization in Eosinophilic Endomyocardial Disease. N Engl J Med [online] 2011 July, 365(2):187-188 [viewed 06 July 2014] Available from: doi:10.1056/NEJMc1103005
  5. AMINI R, NIELSEN C. Eosinophilic myocarditis mimicking acute coronary syndrome secondary to idiopathic hypereosinophilic syndrome: a case report J Med Case Reports [online] :40 [viewed 06 July 2014] Available from: doi:10.1186/1752-1947-4-40
  6. THAMBIDORAI SK, KORLAKUNTA HL, AROUNI AJ, HUNTER WJ, HOLMBERG MJ. Acute Eosinophilic Myocarditis Mimicking Myocardial Infarction Tex Heart Inst J [online] 2009, 36(4):355-357 [viewed 06 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2720293
  7. NIHOYANNOPOULOS P., DAWSON D.. Restrictive cardiomyopathies. European Journal of Echocardiography [online] December, 10(8):iii23-iii33 [viewed 06 July 2014] Available from: doi:10.1093/ejechocard/jep156
  8. MANNELLI LORENZO, CHERIAN VARGHESE, NAYAR AMBIKA, SRICHAI-PARSIA MONVADI. Loeffler's Endocarditis in Hypereosinophilic Syndrome. Current Problems in Diagnostic Radiology [online] 2012 July, 41(4):146-148 [viewed 06 July 2014] Available from: doi:10.1067/j.cpradiol.2011.07.015

Examination

Fact Explanation
Cachexia [1] [2] This is a symptom of hyper eosinophilia, resulting in release of cytokines from eosinophils and causing constitutional symptoms associated with this [1] [2]
Fever [1] [2] This is a symptom of hypereosinophilia, resulting in release of cytokines from eosinophils and causing constitutional symptoms associated with this [1] [2]
Skin rash [1] [2] [8] This is a symptom of hypereosinophilia, resulting in release of cytokines from eosinophils and causing constitutional symptoms associated with this [1] [2]
Increased jugular venous pressure [1] [2] [3] [7] In later stages due to fibrosis of the endocardium and myocardium, there's restrictive cardiomyopathy resulting in bi ventricular failure, a diastolic failure to be exact, hence venous congestion and this. [1] [2] [3] [7]
Dependent oedema [1] [2] [3] [7] In later stages due to fibrosis of the endocardium and myocardium, there's restrictive cardiomyopathy resulting in biventricular failure, a diastolic failure to be exact, hence this. [1] [2] [3] [7]
Hepatomegaly [1] [2] [3] [7] In later stages due to fibrosis of the endocardium and myocardium, there's restrictive cardiomyopathy resulting in biventricular failure, a diastolic failure to be exact, hence venous congestion and this. [1] [2] [3] [7]
Splenomegaly [1] [2] This is a symptom of hypereosinophilia, resulting in release of cytokines from eosinophils and causing constitutional symptoms associated with this [1] [2]
Holo systolic murmur in the apical area [5] Due to myocarditis causing damage to the mitral valve leaflet and mitral regurgitation [5]
Irregular pulse [2] [3] Due to myocarditis, there could be abnormal rhythm generation from the heart causing ventricular arrhythmias. [2] [3]
Retinal lesions [1] [2] [8] This is a symptom of hypereosinophilia, resulting in release of cytokines from eosinophils and causing constitutional symptoms associated with this [1] [2]
Hypotension, cold clammy peripheries [6] This is due to acute myocarditis presenting with cardiogenic shock resulting in inadequate cardiac output, hence hypertension and reduced perfusion hence cold clammy peripheries [6]
References
  1. ACQUATELLA H., SCHILLER N. B., PUIGBO J. J., GOMEZ-MANCEBO J. R., SUAREZ C., ACQUATELLA G.. Value of two-dimensional echocardiography in endomyocardial disease with and without eosinophilia. A clinical and pathologic study. Circulation [online] 1983 June, 67(6):1219-1226 [viewed 05 July 2014] Available from: doi:10.1161/01.CIR.67.6.1219
  2. RIZKALLAH J, DESAUTELS A, MALIK A, ZIEROTH S, JASSAL D, HUSSAIN F, CORDOVA F. Eosinophilic myocarditis: two case reports and review of the literature BMC Res Notes [online] :538 [viewed 06 July 2014] Available from: doi:10.1186/1756-0500-6-538
  3. WRIGHT BENJAMIN L., LEIFERMAN KRISTIN M., GLEICH GERALD J.. Eosinophil Granule Protein Localization in Eosinophilic Endomyocardial Disease. N Engl J Med [online] 2011 July, 365(2):187-188 [viewed 06 July 2014] Available from: doi:10.1056/NEJMc1103005
  4. NIHOYANNOPOULOS P., DAWSON D.. Restrictive cardiomyopathies. European Journal of Echocardiography [online] December, 10(8):iii23-iii33 [viewed 06 July 2014] Available from: doi:10.1093/ejechocard/jep156
  5. TANG A. Severe mitral regurgitation in acute eosinophilic endomyocarditis: repair or replacement?. Interactive Cardiovascular and Thoracic Surgery [online] 2004 June, 3(2):406-408 [viewed 06 July 2014] Available from: doi:10.1016/j.icvts.2004.02.018
  6. SOHN IL SUK, PARK JONG CHUN, CHUNG JAE HUN, KIM KYE HUN, AHN YOUNGKEUN, JEONG MYUNG HO, CHO JEONG GWAN. A Case of Acute Eosinophilic Myopericarditis Presenting with Cardiogenic Shock and Normal Peripheral Eosinophil Count. Korean J Intern Med [online] 2006 December [viewed 06 July 2014] Available from: doi:10.3904/kjim.2006.21.2.136
  7. SPRY CJ. Eosinophils in eosinophilic endomyocardial disease. Postgrad Med J [online] 1986 Jun, 62(728):609-613 [viewed 06 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2418793
  8. MANNELLI LORENZO, CHERIAN VARGHESE, NAYAR AMBIKA, SRICHAI-PARSIA MONVADI. Loeffler's Endocarditis in Hypereosinophilic Syndrome. Current Problems in Diagnostic Radiology [online] 2012 July, 41(4):146-148 [viewed 06 July 2014] Available from: doi:10.1067/j.cpradiol.2011.07.015

Differential Diagnoses

Fact Explanation
Acute myocardial infarction [1] [2] [3] [4] In the early stage of the illness due to necrosis of cardiac myocytes, there could be carditis, resulting in exertiona chest pain with sweating, nausea mimicking acute myocardial infarction [1] [2] [3] [4]
Amyloidosis [5] Systemic amyloidosis is a disorder of protein metabolism in which abnormal extracellular protein material is deposited in organs and tissues. This is the commonest cause of restrictive cardiomyopathy but it can also cause systolic left ventricular dysfunction [5]
Sarcoidosis [5] Sarcoidosis commonly involves the lungs, eyes, and skin but can involve heart as well resulting in granuloma formation. Clinically, cardiac sarcoidosis is manifested as a cardiomyopathy with loss of muscle function or tachyarrhythmias, including heart block, syncope, and sudden death [5]
Iron overload—haemosiderosis [5] Iron loading of tissues is dangerous, because it leads to tissue damage and fibrosis. In the heart, it will lead to myocardial fibrosis and impaired ventricular function with either a restrictive type or dilated cardiomyopathy. [5]
Scleroderma [5] Scleroderma is a multi systemic disease condition that can lead to severe dysfunction and failure of almost any internal organ including the heart and myocardial fibrosis is the hallmark of cardiac involvement in systemic sclerosis [5]
Radiation induced restrictive cardiomyopathy [5] Irradiation of the heart due to the treatment of malignancies can also cause a spectrum of cardiovascular complications including pericarditis, myocardial fibrosis, muscular dysfunction, valvular abnormalities, and conduction disturbances [5]
Idiopathic (primary) restrictive cardiomyopathy [5] This is the diagnosis of restrictive cardiomyopathy using strict haemodynamic criteria which is supported by the absence of specific pathology on either endomyocardial biopsies or evaluation of whole heart specimens. [5]
Viral myocardits [6] Viral myocarditis has been recognized as a cause of congestive heart failure and caused by most commonly adenoviruses and enteroviruses such as the coxsackieviruses. Recently, parvovirus B19 is also recognized. Patients present with fever,constitutional symptoms, chest pain and features of congestive cardiac failure [6]
References
  1. ACQUATELLA H., SCHILLER N. B., PUIGBO J. J., GOMEZ-MANCEBO J. R., SUAREZ C., ACQUATELLA G.. Value of two-dimensional echocardiography in endomyocardial disease with and without eosinophilia. A clinical and pathologic study. Circulation [online] 1983 June, 67(6):1219-1226 [viewed 05 July 2014] Available from: doi:10.1161/01.CIR.67.6.1219
  2. RIZKALLAH J, DESAUTELS A, MALIK A, ZIEROTH S, JASSAL D, HUSSAIN F, CORDOVA F. Eosinophilic myocarditis: two case reports and review of the literature BMC Res Notes [online] :538 [viewed 06 July 2014] Available from: doi:10.1186/1756-0500-6-538
  3. AMINI R, NIELSEN C. Eosinophilic myocarditis mimicking acute coronary syndrome secondary to idiopathic hypereosinophilic syndrome: a case report J Med Case Reports [online] :40 [viewed 06 July 2014] Available from: doi:10.1186/1752-1947-4-40
  4. THAMBIDORAI SK, KORLAKUNTA HL, AROUNI AJ, HUNTER WJ, HOLMBERG MJ. Acute Eosinophilic Myocarditis Mimicking Myocardial Infarction Tex Heart Inst J [online] 2009, 36(4):355-357 [viewed 06 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2720293
  5. NIHOYANNOPOULOS P., DAWSON D.. Restrictive cardiomyopathies. European Journal of Echocardiography [online] December, 10(8):iii23-iii33 [viewed 06 July 2014] Available from: doi:10.1093/ejechocard/jep156
  6. YAJIMA T., KNOWLTON K. U.. Viral Myocarditis: From the Perspective of the Virus. Circulation [online] December, 119(19):2615-2624 [viewed 06 July 2014] Available from: doi:10.1161/​CIRCULATIONAHA.108.766022

Investigations - for Diagnosis

Fact Explanation
Full blood count [1] Performed to look for the presence of eosinophils. Note that Peripheral eosinophilia isn't mandatory for the diagnosis of Loeffler endocarditis. [1]
Cytogenetics, fluorescent in situ hybridization (FISH), and molecular analysis [2] This can show the presence of the FIP1L1-PDGFRA fusion gene [2]
Electrocardiogram [3] [4] [8] This will show nonspecific ST-segment and T-wave abnormalities and arrhythmias including atrial fibrillation and ventricular arrhythmias. [3] [4]
Echocardiography [5] [6] [8] Shows a restrictive pattern filling with left ventricular systolic function preserved and valvular regurgitaion and apical thrombus in the left ventricle also has been reported [5] [6] [7]
Doppler studies using echocardiogram [10] This will show restrictive cardiomyopathy [10]
cardiac catheterization [9] May show severely elevated pressure in ventricular filling and the presence of tricuspid or mitral regurgitation. [9]
endomyocardial biopsy [1] [8] This remains the investigation of choice and histologic specimens show thick and deep layers of loosely arranged collagen tissue localized to the endocardium primarily, they may have strands extending into the myocardium underlying as well and eosinophilic infiltration which is not commonly seen. Also it may show extensive interstitial edema associated with necrosis of myocytes, diffuse infiltration of inflammatory cells with lymphocytes, neutrophils [1] [8]
MRI of the chest [5] [9] Right ventricular mild systolic and diastolic dysfunction and extensive subendocardial delayed contrast enhancement suggests endomyocardial fibrosis [5] [9]
Nuclear imaging [5] This is done with gallium67- or indium111-labeled antimyosin antibodies can be useful in assessing myocardial dysfunction but there are no specific features that help establish the diagnosis [5]
References
  1. HAJSADEGHI SHOKOUFEH, CHITSAZAN MITRA, POURALIAKBAR HAMID REZA, SADEGHIPOUR ALIREZA. From an isolated right ventricular thrombus to the diagnosis of the hypereosinophilic syndrome. Journal of Cardiology Cases [online] 2011 June, 3(3):e133-e136 [viewed 05 July 2014] Available from: doi:10.1016/j.jccase.2011.03.002
  2. SHAH SHILPAN, LOGHAVI SANAM, GARCIA-MANERO GUILLERMO, KHOURY JOSEPH D. Discovery of imatinib-responsive FIP1L1-PDGFRA mutation during refractory acute myeloid leukemia transformation of chronic myelomonocytic leukemia. Array [online] 2014 December [viewed 06 July 2014] Available from: doi:10.1186/1756-8722-7-26
  3. AMINI R, NIELSEN C. Eosinophilic myocarditis mimicking acute coronary syndrome secondary to idiopathic hypereosinophilic syndrome: a case report J Med Case Reports [online] :40 [viewed 06 July 2014] Available from: doi:10.1186/1752-1947-4-40
  4. THAMBIDORAI SK, KORLAKUNTA HL, AROUNI AJ, HUNTER WJ, HOLMBERG MJ. Acute Eosinophilic Myocarditis Mimicking Myocardial Infarction Tex Heart Inst J [online] 2009, 36(4):355-357 [viewed 06 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2720293
  5. RIZKALLAH J, DESAUTELS A, MALIK A, ZIEROTH S, JASSAL D, HUSSAIN F, CORDOVA F. Eosinophilic myocarditis: two case reports and review of the literature BMC Res Notes [online] :538 [viewed 06 July 2014] Available from: doi:10.1186/1756-0500-6-538
  6. NIHOYANNOPOULOS P., DAWSON D.. Restrictive cardiomyopathies. European Journal of Echocardiography [online] December, 10(8):iii23-iii33 [viewed 06 July 2014] Available from: doi:10.1093/ejechocard/jep156
  7. TANG A. Severe mitral regurgitation in acute eosinophilic endomyocarditis: repair or replacement?. Interactive Cardiovascular and Thoracic Surgery [online] 2004 June, 3(2):406-408 [viewed 06 July 2014] Available from: doi:10.1016/j.icvts.2004.02.018
  8. SOHN IS, PARK JC, CHUNG JH, KIM KH, AHN Y, JEONG MH, CHO JG. A Case of Acute Eosinophilic Myopericarditis Presenting with Cardiogenic Shock and Normal Peripheral Eosinophil Count Korean J Intern Med [online] 2006 Jun, 21(2):136-140 [viewed 06 July 2014] Available from: doi:10.3904/kjim.2006.21.2.136
  9. LANGWIESER N., VON OLSHAUSEN G., RISCHPLER C., IBRAHIM T.. Confirmation of diagnosis and graduation of inflammatory activity of Loeffler endocarditis by hybrid positron emission tomography/magnetic resonance imaging. European Heart Journal [online] 2014 April [viewed 06 July 2014] Available from: doi:10.1093/eurheartj/ehu148
  10. SEO JS, SONG JM, KIM DH, KANG DH, SONG JK. A Case of Loeffler's Endocarditis Associated with Churg-Strauss Syndrome J Cardiovasc Ultrasound [online] 2010 Mar, 18(1):21-24 [viewed 06 July 2014] Available from: doi:10.4250/jcu.2010.18.1.21

Investigations - Fitness for Management

Fact Explanation
Full blood count [3] This is done to exclude significant anaemia and any quantitative platelet or leukocyte abnormality [3]
Serum Creatinine and Blood urea nitogen, and serum electrolyes [3] As these patients are on chronic diuretic therapy it may produce total-body sodium and potassium depletion, and uraemia. Hypokalaemia is a relatively common finding in cardiac surgical patients. Also to assess renal function [3]
Coagulation profile [3] To exclude any coagulopathy, and also specially since patients are on Warfarin this is important [3]
Echocardiography [1] [2] To evaluate the ejection fraction, and valvular regurgitation prior to cardiac surgery [1] [2]
Cardio-pulmonary exercise testing [1] [2] To evaluate the cardiac function before cardiac surgery [1] [2]
References
  1. SCHWAIBLMAIR MARTIN, REICHENSPURNER HERMANN, MÜLLER CHRISTIAN, BRIEGEL JOSEF, FÜRST HEINER, GROH JÜRGEN, REICHART BRUNO, VOGELMEIER CLAUS. Cardiopulmonary Exercise Testing Before and After Lung and Heart–Lung Transplantation. Am J Respir Crit Care Med [online] 1999 April, 159(4):1277-1283 [viewed 06 July 2014] Available from: doi:10.1164/ajrccm.159.4.980511
  2. STRUTHERS R., ERASMUS P., HOLMES K., WARMAN P., COLLINGWOOD A., SNEYD J. R.. Assessing fitness for surgery: a comparison of questionnaire, incremental shuttle walk, and cardiopulmonary exercise testing in general surgical patients. British Journal of Anaesthesia [online] December, 101(6):774-780 [viewed 06 July 2014] Available from: doi:10.1093/bja/aen310
  3. CORNELISSEN H.. Preoperative assessment for cardiac surgery. Continuing Education in Anaesthesia, Critical Care & Pain [online] 2006 June, 6(3):109-113 [viewed 06 July 2014] Available from: doi:10.1093/bjaceaccp/mkl013

Investigations - Followup

Fact Explanation
full blood count [1] In patient serial full blood counts are done to assess peripheral eosinophilia. [1]
Echocardiography [1] [2] [3] Serial echocardiographic investigations are done to evaluate ejection fraction, diastolic dysfunction and response to treatment. [1][2] [3]
Serum electrolytes [4] Since patients are put on diuretics for congestive cardiac failure and angiotensinogen converting enzyme inhibitors it's important to monitor serum electrolytes [4]
Liver function tests [5] As patients are put on immunosuppressants, it's important to monitor liver function tests to detect drug induced side effects [5]
Prothrombin time and international normalization ratio [6] As patients are put on Warfarin to combat thrombo-embolism, it's important to monitor this [6]
References
  1. RIZKALLAH J, DESAUTELS A, MALIK A, ZIEROTH S, JASSAL D, HUSSAIN F, CORDOVA F. Eosinophilic myocarditis: two case reports and review of the literature BMC Res Notes [online] :538 [viewed 06 July 2014] Available from: doi:10.1186/1756-0500-6-538
  2. NIHOYANNOPOULOS P., DAWSON D.. Restrictive cardiomyopathies. European Journal of Echocardiography [online] December, 10(8):iii23-iii33 [viewed 06 July 2014] Available from: doi:10.1093/ejechocard/jep156
  3. TANG A. Severe mitral regurgitation in acute eosinophilic endomyocarditis: repair or replacement?. Interactive Cardiovascular and Thoracic Surgery [online] 2004 June, 3(2):406-408 [viewed 06 July 2014] Available from: doi:10.1016/j.icvts.2004.02.018
  4. Part 10.1: Life-Threatening Electrolyte Abnormalities. Circulation [online] 2005 November, 112(24_suppl):IV-121-IV-125 [viewed 06 July 2014] Available from: doi:10.1161/​CIRCULATIONAHA.105.166563
  5. AGGARWAL V., WILLIAMS M D, BEATH S V. Gastrointestinal problems in the immunosuppressed patient. Archives of Disease in Childhood [online] 1998 January, 78(1):5-8 [viewed 06 July 2014] Available from: doi:10.1136/adc.78.1.5
  6. FIUMARA K., GOLDHABER S. Z.. A Patient's Guide to Taking Coumadin/Warfarin. Circulation [online] 2009 March, 119(8):e220-e222 [viewed 06 July 2014] Available from: doi:10.1161/​CIRCULATIONAHA.108.803957

Investigations - Screening/Staging

Fact Explanation
Cytogenetics, fluorescent in situ hybridization (FISH), and molecular analysis [2] This can show the presence of the FIP1L1-PDGFRA fusion gene [2]
References
  1. SHAH SHILPAN, LOGHAVI SANAM, GARCIA-MANERO GUILLERMO, KHOURY JOSEPH D. Discovery of imatinib-responsive FIP1L1-PDGFRA mutation during refractory acute myeloid leukemia transformation of chronic myelomonocytic leukemia. Array [online] 2014 December [viewed 06 July 2014] Available from: doi:10.1186/1756-8722-7-26

Management - General Measures

Fact Explanation
Patient education [1] [2] [3] Patient should be educated on the aetiology of the disease, the course, importance of follow up, the advices regarding Warfarin and other medication, diet and exercise [1]
Management of heart failure with pharmacological therapy [4] [6] Patients are put on heart failure regime once the heart failure ensues. Diuretics, Digoxin, After-load reduction with angiotensinogen converting enzyme inhibitors are used [4]
Anticoagulant therapy [5] Anticoagulant therapy is started with Warfarin to combat thrombo-embolism related to cardiac dysfunction. [5]
Diet [2] [3] Special restrictions in the diet is unnecessary except for low salt diet in congestive cardiac failure [2]
Exercise [2] [3] Strenuous exercises are avoided and moderate endurance aerobic activities are advised. [2]
References
  1. FIUMARA K., GOLDHABER S. Z.. A Patient's Guide to Taking Coumadin/Warfarin. Circulation [online] 2009 March, 119(8):e220-e222 [viewed 06 July 2014] Available from: doi:10.1161/​CIRCULATIONAHA.108.803957
  2. MOSER D. K.. Improving Outcomes in Heart Failure: It's Not Unusual Beyond Usual Care. [online] 2002 June, 105(24):2810-2812 [viewed 06 July 2014] Available from: doi:10.1161/​01.CIR.0000021745.45349.BB
  3. JEON YUN-HEE, KRAUS STEFAN G, JOWSEY TANISHA, GLASGOW NICHOLAS J. The experience of living with chronic heart failure: a narrative review of qualitative studies. Array [online] 2010 December [viewed 06 July 2014] Available from: doi:10.1186/1472-6963-10-77
  4. GILLESPIE N. D. The diagnosis and management of chronic heart failure in the older patient. British Medical Bulletin [online] 2006 February, 75-76(1):49-62 [viewed 06 July 2014] Available from: doi:10.1093/bmb/ldh060
  5. DEDIEU N., GIARDINI A., KHAMBADKONE S., MAREK J.. Eosinophilic heart disease in a paediatric patient. European Journal of Echocardiography [online] December, 12(1):E3-E3 [viewed 06 July 2014] Available from: doi:10.1093/ejechocard/jeq098
  6. SEO JS, SONG JM, KIM DH, KANG DH, SONG JK. A Case of Loeffler's Endocarditis Associated with Churg-Strauss Syndrome J Cardiovasc Ultrasound [online] 2010 Mar, 18(1):21-24 [viewed 06 July 2014] Available from: doi:10.4250/jcu.2010.18.1.21

Management - Specific Treatments

Fact Explanation
Pharamacological therapy with Corticosteroids [1] [2] [4] [5] [7] [8] Corticosteroids is used in acute myocarditis, and may prolong survival as well. [1] [2] [4] [5] [7] [8]
Pharamacological therapy with cytotoxic drugs, including hydroxyurea [1] [4] Early phases of the disease can be treated with immune suppressant and cytotoxic medications, and thought to increase survival as well [1]
Pharamacological therapy with tyrosine-kinase inhibitors [9] Treatment with low-dose imatinib, which is Tyrosine-Kinase inhibitor cause rapid regression of both eosinophilic proliferation and endomyocardiopathy [9]
Pharamacological therapy with interferons [1] [3] Interferon alpha appears to be of some success in reducing eosinophilic degranulation [1] [3]
Surgical treatment with endocardiectomy [4] Directed toward the predominant location of the restrictive process and is done when restrictive process is so advanced, especially in patients with congestive heart failure refractory to medical therapy [4]
Surgical treatment with valve replacement or repair [6] The mitral and tricuspid valves which are damaged during the disease process can be replaced or repair [6]
Heart transplant [4] When other surgical procedures limit the success, heart transplantation remains the last choice [4]
References
  1. SEO JS, SONG JM, KIM DH, KANG DH, SONG JK. A Case of Loeffler's Endocarditis Associated with Churg-Strauss Syndrome J Cardiovasc Ultrasound [online] 2010 Mar, 18(1):21-24 [viewed 06 July 2014] Available from: doi:10.4250/jcu.2010.18.1.21
  2. RIZKALLAH J, DESAUTELS A, MALIK A, ZIEROTH S, JASSAL D, HUSSAIN F, CORDOVA F. Eosinophilic myocarditis: two case reports and review of the literature BMC Res Notes [online] :538 [viewed 06 July 2014] Available from: doi:10.1186/1756-0500-6-538
  3. WRIGHT BENJAMIN L., LEIFERMAN KRISTIN M., GLEICH GERALD J.. Eosinophil Granule Protein Localization in Eosinophilic Endomyocardial Disease. N Engl J Med [online] 2011 July, 365(2):187-188 [viewed 06 July 2014] Available from: doi:10.1056/NEJMc1103005
  4. AMINI R, NIELSEN C. Eosinophilic myocarditis mimicking acute coronary syndrome secondary to idiopathic hypereosinophilic syndrome: a case report J Med Case Reports [online] :40 [viewed 06 July 2014] Available from: doi:10.1186/1752-1947-4-40
  5. THAMBIDORAI SK, KORLAKUNTA HL, AROUNI AJ, HUNTER WJ, HOLMBERG MJ. Acute Eosinophilic Myocarditis Mimicking Myocardial Infarction Tex Heart Inst J [online] 2009, 36(4):355-357 [viewed 06 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2720293
  6. TANG A. Severe mitral regurgitation in acute eosinophilic endomyocarditis: repair or replacement?. Interactive Cardiovascular and Thoracic Surgery [online] 2004 June, 3(2):406-408 [viewed 06 July 2014] Available from: doi:10.1016/j.icvts.2004.02.018
  7. SOHN IS, PARK JC, CHUNG JH, KIM KH, AHN Y, JEONG MH, CHO JG. A Case of Acute Eosinophilic Myopericarditis Presenting with Cardiogenic Shock and Normal Peripheral Eosinophil Count Korean J Intern Med [online] 2006 Jun, 21(2):136-140 [viewed 06 July 2014] Available from: doi:10.3904/kjim.2006.21.2.136
  8. SPRY CJ. Eosinophils in eosinophilic endomyocardial disease. Postgrad Med J [online] 1986 Jun, 62(728):609-613 [viewed 06 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2418793
  9. REUSS C. S., WILANSKY S.. Eosinophilic Heart Disease in Acute Myeloproliferative Disorder. Circulation [online] 2007 June, 115(23):e614-e616 [viewed 06 July 2014] Available from: doi:10.1161/​CIRCULATIONAHA.107.692574