The salient features of this condition are present in the case presented here of a 14-year-old boy who died from progressive heart failure due to right ventricular EMF. [ncbi.nlm.nih.gov]

• Swelling

  A few weeks later, she arrived in the US and was admitted for worsening decompensated HF with symptoms of fatigue, shortness of breath and abdominal distention out of proportion to lower extremity swelling. [jacc.org]

  The final case was a 27-year-old farmer with cough, progressive shortness of breath and abdominal swelling. [nigjcardiol.org]

  Facial swelling (edema), swelling of the legs, enlargement of the spleen and liver (hepatosplenomegaly), and an accumulation of fluid in the abdominal cavity (ascites) are to be expected. [rarediseases.org]

• Congestive Heart Failure

  Full Text A 40-year-old male with an implanted pacemaker, who had lived in a tropical country for more than 15 years, was admitted because of congestive heart failure. [revportcardiol.org]

  Physical signs in patients are dominated by features of congestive heart failure. [link.springer.com]

• Weakness

  IgM reactivity was weak in both groups, although higher in EMF patients (11/56; 19.6%) when compared to controls (n = 0). [ncbi.nlm.nih.gov]

  A 59-year-old woman was examined to investigate weakness, fatigue, and abdominal pain. Clinical examination revealed vitiligo, raised jugular venous pressure, bilateral pleural effusion, splenomegaly, and mild peripheral edema. [ajronline.org]

  Weak apex beat in 5th intercostal space along mid-axillary line. Much increased area of precordial dullness. Well marked triple rhythm. S3 heard all over. Accentuated pulmonary second sound. Systolic murmur. [scitcentral.com]

  Most of the patients of endomyocardial fibrosis present with symptoms of heart failure, generalized weakness, and fever. [heartviews.org]

• Weight Loss

  The presenting symptoms of IHES are nonspecific and include weight loss, abdominal pain, rash, and congestive heart failure [1]. [ajronline.org]

  EMF can easily be confused with other common causes of ascites and weight loss in children such as chronic liver disease and tuberculosis especially where the tuberculosis score is routinely applied, as was the case in this patient; therefore, it should [amhsr.org]

  Cough was productive of copious non-foul-smelling sputum, occasionally blood stained, associated with breathlessness, intermittent low grade fever, anorexia and progressive weight loss. He had a history of significant alcohol use and smoking. [nigjcardiol.org]

• Sepsis

  Four months later, she was admitted with fever and signs of sepsis, and died soon after. [ncbi.nlm.nih.gov]

  Four patients died of heart failure and 1 of overimposed sepsis. Surgery was successful in 2 patients with the biventricular form of the disease. [onlinejase.com]

• Splenomegaly

  Thoraco-abdominal CT confirmed hepato- and splenomegaly and showed mild calcification in the left ventricular apex ( Figure 1 A, arrow). Given the suspicion of EF, the patient was referred for echocardiographic assessment ( Figure 1 B). [revportcardiol.org]

  Clinical examination revealed vitiligo, raised jugular venous pressure, bilateral pleural effusion, splenomegaly, and mild peripheral edema. [ajronline.org]

  Effects of cassava diet on cercopithecusAethiops Livers: a case for cassavas the cause of both tropical splenomegaly syndrome (tss) and endomyocardial fibrosis (EMF). East Afr Med J. 1996;73(5, Suppl):S24-8. [PubMed] [Google Scholar] 15. [ncbi.nlm.nih.gov]

  Right ventricular EMF may present with chronic systemic venous hypertension leading to elevated jugular pressure, gross hepatomegaly and congestive splenomegaly. 8 Left ventricular EMF may present with a loud left ventricular third heart sound, short [bjcardio.co.uk]

• Pneumonia

  Other remarkable features were the presence of a right ventricular diverticulum, and a positive Mycoplasma pneumoniae immunoglobulin M enzyme-linked immunosorbent assay test. [ncbi.nlm.nih.gov]

  The lung showed patchy edema and interstial pneumonia; a single localized area of eosinophilic granuloma was identified. Gynaecomastia was present. Apart from autolysis and congestion, the pancreas, thyroid and kidney revealed nothing noteworthy. [scitcentral.com]

  The newborn required orotracheal intubation in the sixth hour of life, when pneumonia was diagnosed, and he remained under mechanical ventilation for 7 days. [scielo.br]

  Hypothesis References Disproving the Hypothesis Eosinophilia and parasitic disease Eosinophilia 22, 47–52, 54–56, 63, 75–78, 106 109 Immuno-allergy inducers 56, 59, 60 Schistosomiasis 54, 55, 71 30, 65 Filariasis 47, 75–77, 106 29 Virus, myocoplasma pneumoniae [ahajournals.org]

• Orthopnea

  September 2017, Vol. 117, 601. doi:10.7556/jaoa.2017.115 The Journal of the American Osteopathic Association, September 2017, Vol. 117, 601. doi:10.7556/jaoa.2017.115 A 65-year-old man presented to a medical center with dyspnea, peripheral edema, and orthopnea [jaoa.org]

  Fatigue, cachexia, and orthopnea are also commonly present. Mitral regurgitation can occur due to the fibrotic involvement of the chordae tendineae and posterior mitral valve leaflet. [statpearls.com]

  However, when the left ventricle is affected, dyspnea, exertional dyspnea, orthopnea, nocturnal paroxysmal dyspnea, and fatigue will predominate. [ncbi.nlm.nih.gov]

• Pleural Effusion

  A triphasic abdominal CT scan (not shown) revealed a nonenhancing thrombus in both ventricular cavities, a small pericardial effusion, and bilateral pleural effusion. [ajronline.org]

  Cine imaging ( C, Supplementary data online, Video S1 ) showed pericardial and pleural effusion, biventricular thickening and systolic dysfunction. [academic.oup.com]

  effusions and/or fibrosis, [6] and neurological tests such as CT scans can show strokes and increased cerebrospinal fluid pressure. [6] A proportion of patients have a mutation involving the PDGFRA and FIP1L1 genes on the fourth chromosome, leading to [en.wikipedia.org]

  Chest radiography revealed left pleural effusion and mild cardiomegaly. Pleural fluid from thoracentesis was negative for malignancy. [mayoclinicproceedings.org]

  Common abnormalities seen on the chest radiograph include cardiomegaly, interstitial edema, pleural effusion(s), and evidence of previous sternotomy (sternal wires). [clevelandclinicmeded.com]

• Abdominal Pain

  The presenting symptoms of IHES are nonspecific and include weight loss, abdominal pain, rash, and congestive heart failure [1]. [ajronline.org]

  pain, nausea, hyperthermia, and marked abdominal spasm and tenderness at palpation. [academic.oup.com]

  Abdominal distension with abdominal pain was seen in all patients. Two patients had abdominal distension without fluid. All others had ascites. Nineteen patients had a pericardial effusion (53%). [southsudanmedicaljournal.com]

  Hospitalized four times, he was operated on for abdominal pains. No major illness in childhood, playing normally. On examination, he was emaciated. Ascites. ++ Leg edema +. JVP over 10 cm. Irregular fibrillating pulse. Normotensive. [scitcentral.com]

• Heart Failure

  Endomyocardial fibrosis is a rarely seen complication of Behçet's disease leading to progressive heart failure. [ncbi.nlm.nih.gov]

  Am Heart J. 1973; 85: 308 Scopus (131) Abstract Full Text PDF Google Scholar Perloff JK Lindgren KM Groves BM Uncommon or commonly unrecognized causes of heart failure. in: Friedberg CK Congestive Heart Failure. [ajconline.org]

• Heart Disease

  We report this rare case of mitral valve prolapse in EMF, in a geographical area where rheumatic heart disease is endemic, to showcase how a rare manifestation of EMF can be misdiagnosed as that of rheumatic heart disease. [ncbi.nlm.nih.gov]

  Rheumatic heart disease and endomyocardial fibrosis: Distinguishing the etiology of mitral regurgitation in low-resourced areas Abstract Rheumatic heart disease (RHD) and endomyocardial fibrosis (EMF) are 2 neglected cardiovascular diseases that disproportionately [journals.ac.za]

  Endocardial fibroelastosis; a factor in heart disease of obscure etiology. New England J. Med. 1954; 251: 327 Scopus (18) PubMed Crossref Google Scholar Auld W.H.R. Watson H. Fibroelastosis of the heart in adolescence. Brit. [amjmed.com]

  Br Heart J. 1989; 62: 450-454 Freers J. Mayanja-Kizza H. Ziegler J.L. et al. Echocardiographic diagnosis of heart disease in Uganda. Trop Doct. 1996; 26: 125-128 Seth S. Thatai D. Sharma S. et al. [jcvaonline.com]

  Clinical features depend on stage of disease and anatomical involvement of the heart. [nigjcardiol.org]

• Mitral Valve Prolapse

  Mitral valve prolapse in endomyocardial fibrosis (EMF) is an unusual entity. Literature search reveals only 1 report of mitral valve prolapse assosiated with EMF. [ncbi.nlm.nih.gov]

  Valve Prolapse in Endomyocardial Fibrosis. ( 27549547 ) Xavier J....Suryaprakash S. 2016 26 Doughnut appearance of left ventricle in isolated left ventricular endomyocardial fibrosis. ( 27751273 ) Saran M....Sivasubramonian S. 2016 27 Letter by Kothari [malacards.org]

  […] regurgitation (MR)僧帽弁逆流［症］ mitral stenosis (MS) 僧帽弁狭窄［症］ mitral valve 僧帽弁 mitral valve area僧帽弁口面積 mitral valve prolapse (MVP)僧帽弁逸脱［症］ mitral valve replacement僧帽弁置換術 Mobitz type I blockモビッツI型ブロック Mobitz type II blockモビッツII型ブロック moist rale湿性ラ音 mucocutaneous [tokyo-med.ac.jp]

• Continuous Murmur

  […] complete transposition of great arteries完全大血管転位［症］ complication 合併症 composite graft人工弁逢着人工血管 concentric hypertrophy求心性心肥大 conduction 伝導 conduit導管 congenital heart disease (CHD) 先天性心疾患 congestive heart failure (CHF)うっ血性心不全 constrictive pericarditis 収縮性心膜炎 continuous [tokyo-med.ac.jp]

• Prolapse

  Mitral valve prolapse in endomyocardial fibrosis (EMF) is an unusual entity. Literature search reveals only 1 report of mitral valve prolapse assosiated with EMF. [ncbi.nlm.nih.gov]

  Transthoracic echocardiography revealed prolapse of the anterior mitral leaflet with severe (4+) mitral regurgitation, enlarged left atrium (50 mm), and dilated left ventricle (46 mm at end-diastole and 26 mm at end-systole). [jtcvs.org]

  29364349 ) 2017 23 Endomyocardial fibrosis in Sudan: clinical and echocardiographic features. ( 28906536 ) 2017 24 Rare association of endomyocardial fibrosis and Chagas heart disease. ( 28031230 ) Hotta V.T....Mady C. 2016 25 A Rare Case of Mitral Valve Prolapse [malacards.org]

  […] equivalents (MET[s])メッツ、代謝率 mismatch乘離 mitral annular (ring) calcification僧帽弁輪部石灰化 mitral commissurotomy僧帽弁交連切開［術］ mitral opening snap僧帽弁開放音 mitral regurgitation (MR)僧帽弁逆流［症］ mitral stenosis (MS) 僧帽弁狭窄［症］ mitral valve 僧帽弁 mitral valve area僧帽弁口面積 mitral valve prolapse [tokyo-med.ac.jp]

• Skin Rash

  Sarcoidosis may be suspected in patients with cardiomyopathy and lymphadenopathy, skin rashes, or splenomegaly. [clevelandclinicmeded.com]

• Suggestibility

  Suggested Listings for Evaluation: DETERMINATION LISTING REMARKS Meets 4.02 4.05 Equals * Adjudicators may, at their discretion, use the Medical Evidence of Record or the listings suggested to evaluate the claim. [secure.ssa.gov]

  Suggested Citation: Suggested Citation Puvaneswary, Murugasu and Joshua, Fred and Ratnarajah, Siva, Idiopathic Hypereosinophilic Syndrome: Magnetic Resonance Imaging Findings in Endomyocardial Fibrosis (2001-05-23). [papers.ssrn.com]

  Anatomicopathological and surgical findings suggested endomyocardial fibrosis, although that pathology is very rare at the patient's age. [scielo.br]

  The afore going composition, found to be non-homologous to all molecules of chemical species in the databases searched, suggests the possible role of a metabolic pathway in the pathogenesis of EMF if aligned with our "molecular mimicry" hypothesis. [ncbi.nlm.nih.gov]

European citizens with no history of travelling to tropical countries.3 Some of these reports have communicated severely calcified forms of this disease.4 We would like to state that an exhaustive evaluation was performed to complete the diagnostic workup [revespcardiol.org]

• Pericardial Effusion

  X-ray features were typical of pericardial effusion masking underlying endomyocardial fibrosis. Endomyocardial fibrosis is a neglected research field. It needs more attention from biomedical researchers. [ncbi.nlm.nih.gov]

  They may also have pericardial effusion, marked cardiomegally, RV third heart sound, and inconspicuous systolic murmur of TR. [nigjcardiol.org]

  A large pericardial effusion is often present. The right atrium may be enormously dilated. [slideshare.net]

• Myocardial Fibrosis

  A 50-year-old female patient with endo- myocardial fibrosis presented with unusual calcifications of the right ventricular endocardium that embedded the tricuspid valve papillary muscles. [ncbi.nlm.nih.gov]

  Finally, on cardiac MRI subendocardial late gadolinium hyperenhancement was observed in the left ventricular apex ( Figure 1 D, arrow), reflecting myocardial fibrosis. The patient's clinical context supports the diagnosis of an initial stage of EF. [revportcardiol.org]

  fibrosis and focal myocardial fibrosis. [wikidoc.org]

  Myocardial fibrosis consists of collagen deposition and fibroblast proliferation. These changes can potentially explain most of the symptoms in patients with EMF. [emedicine.medscape.com]

• Abnormal ECG

  Common ECG findings of EMF include P-wave abnormalities, reduced QRS voltages, and flattened or inverted T-waves. 10 TTE was previously the primary imaging tool for identifying EMF. [bjcardio.co.uk]

• Pleural Effusion

  A triphasic abdominal CT scan (not shown) revealed a nonenhancing thrombus in both ventricular cavities, a small pericardial effusion, and bilateral pleural effusion. [ajronline.org]

  Cine imaging ( C, Supplementary data online, Video S1 ) showed pericardial and pleural effusion, biventricular thickening and systolic dysfunction. [academic.oup.com]

  effusions and/or fibrosis, [6] and neurological tests such as CT scans can show strokes and increased cerebrospinal fluid pressure. [6] A proportion of patients have a mutation involving the PDGFRA and FIP1L1 genes on the fourth chromosome, leading to [en.wikipedia.org]

  Chest radiography revealed left pleural effusion and mild cardiomegaly. Pleural fluid from thoracentesis was negative for malignancy. [mayoclinicproceedings.org]

  Common abnormalities seen on the chest radiograph include cardiomegaly, interstitial edema, pleural effusion(s), and evidence of previous sternotomy (sternal wires). [clevelandclinicmeded.com]

Surgical treatment of endomyocardial fibrosis. [jtcvs.org]

Acute eosinophilic myocarditis: diagnosis and treatment. Acute Card Care. 2010; 12 (1):31-36. doi: 10.3109/17482940903578998 [CrossRef] [PubMed] 3. Kawano S, Kato J, Kawano N, et al. [jaoa.org]

Outcome was favorable under treatment with prednisone and azathioprine.This case illustrates a favorable outcome of endomyocardial fibrosis during pregnancy. [ncbi.nlm.nih.gov]

Endomyocardial fibrosis carries a poor prognosis, due to the late presentation of patients. [ncbi.nlm.nih.gov]

Various infections and toxic factors were postulated regarding its etiology. [scopemed.org]

Endomyocardial fibrosis (EMF) is a rare progressive restrictive cardiomyopathy of unknown etiology that mainly presents with heart failure. [ncbi.nlm.nih.gov]

Due to lack of resources for research in the disease endemic areas, the exact epidemiology, etiology and pathogenesis remain unknown, and the natural history is incompletely understood. [scopemed.org]

However, due to lack of resources for research in these areas, the exact epidemiology, etiology and pathogenesis remain unknown, and the natural history is incompletely understood. [globalcardiologyscienceandpractice.com]

An epidemiologic, clinical, and pathologic study. Am Heart J. 1967; 74: 687-709 Connor DH Somers K. Hutt MSR Manion WC D’Arbela PG Endomyocardial fibrosis in Uganda (Davies’ disease). Part II. An epidemiologic, clinical, and pathologic study.. [journal.chestnet.org]

An epidemiologic, clinical, and pathologic study. Am Heart J 74 : 687–709 3. Sliwa K et al. (2005) Epidemiology and etiology of cardiomyopathy in Africa. Circulation 112 : 3577–3583 4. [nature.com]

The pathophysiological hallmark of the disease is focal or diffuse endocardial thickening involving mainly the inflow, the apices, and the subvalvular region leading to valvular regurgitation, diastolic dysfunction and obliteration of the ventricular [ncbi.nlm.nih.gov]

Postgrad Med J. 1983; 59: 179-183 Scopus (24) PubMed Crossref Google Scholar Fauci AS The idiopathic hypereosinophilic syndrome: clinical, pathophysiologic and therapeutic considerations. [internationaljournalofcardiology.com]

Pathophysiology EMF involves both the right and left ventricle in 50% of cases. Forty percent of cases have solely the left ventricle involved, and in the remaining 10% of cases, the right ventricle is involved. [statpearls.com]

PubMed Google Scholar, 4 Fauci AS Harley JB Roberts WC Ferrans VJ Gralnick HR Bjornson BH The idiopathic hypereosinophilic syndrome: clinical, pathophysiologic, and therapeutic considerations. [mayoclinicproceedings.org]

CONCLUSION: Short term prednisolone use was generally safe and did not prevent reaccumulation of ascites in this study population. TRIAL REGISTRATION NUMBER: ISRCTN63999319, 28/03/2013. [ncbi.nlm.nih.gov]

If diagnosed early, at the time of myocarditis, treatment with immunosuppressive therapies may prevent disease progression. 14,15 If the disease develops to a restrictive cardiomyopathy, the opportunity to prevent fibrosis recedes and management shifts [bjcardio.co.uk]