Presentation
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]
Entire Body System
- Splenectomy
Her past medical history was remarkable for paroxysmal supraventricular arrhythmia, hypothyroidism, a previous diagnosis of Wherlof Disease treated with splenectomy, chronic obstructive pulmonary disease treated with inhaled corticosteroids and moderate [heartlungcirc.org]
[…] eosinophil levels and preventing further damage to organs. [6] Corticosteroids, such as prednisone, are good for reducing eosinophil levels and antineoplastics are useful for slowing eosinophil production. [6] Surgical therapy is rarely utilised, however splenectomy [en.wikipedia.org]
Respiratoric
- 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]
- Aspiration
A haematologic consultation was made in order to assess the opportunity of a bone-marrow aspiration for ruling out a myeloproliferative disorder or a hypereosinophilic syndrome with clonal aetiology but considering the only mildly elevated eosinophils [heartlungcirc.org]
Cardiovascular
- 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]
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]
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]
Clinical features depend on stage of disease and anatomical involvement of the heart. [nigjcardiol.org]
- Nocturnal Paroxysmal Dyspnea
However, when the left ventricle is affected, dyspnea, exertional dyspnea, orthopnea, nocturnal paroxysmal dyspnea, and fatigue will predominate. [ncbi.nlm.nih.gov]
- 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]
- Heart Murmur
We noted a muffling of heart sounds in 24 patients. Heart murmurs were noted in 22 patients. [annalspc.com]
Psychiatrical
- 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]
Urogenital
- Renal Insufficiency
The patient's medical history was notable for coronary artery disease, hypertension, hyperlipidemia, type 2 diabetes mellitus, chronic renal insufficiency, gout, and anemia. [mayoclinicproceedings.org]
Workup
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]
P Wave
- P Mitrale
The ECG showed sinus rhythm, P mitrale, deep S waves in V3 and pronounced R waves in V4, profound ST depression and deep T wave inversion in V3-6, without criteria for LVH (Fig 2A). [escardio.org]
Other Pathologies
- 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]
Treatment
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]
Prognosis
Endomyocardial fibrosis carries a poor prognosis, due to the late presentation of patients. [ncbi.nlm.nih.gov]
Etiology
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]
Epidemiology
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]
Pathophysiology
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]
Prevention
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]