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Endomyocardial Fibrosis

Endomyocardial Fibroses


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]
  • Notwithstanding attempts to standardize the diagnostic criteria, the clinical presentation may still be challenging.[ncbi.nlm.nih.gov]
  • We present one such case of endomyocardial fibrosis, in which a large thrombus was seen adherent to the anterolateral wall of right atrium, posing further risk of thromboembolism with complex management issues.[ncbi.nlm.nih.gov]
  • This condition is not well understood, with varied manifestations, from subclinical presentations to chronic and progressive edematous syndromes.[ncbi.nlm.nih.gov]
  • Left ventricular endomyocardial fibrosis presents with severe pulmonary hypertension and right ventricular endomyocardial fibrosis presents very high systemic venous pressure and congestive cardiac failure.[ncbi.nlm.nih.gov]
Turkish
  • We report a case of right ventricular endomyocardial fibrosis mimicking Ebstein anomaly in a 26-year-old male Turkish patient with Behçet's disease, who had heart failure symptoms.[ncbi.nlm.nih.gov]
Sputum
  • Case 3 TJ, a 27-year-old male farmer presented with 1-year history of abdominal swelling, progressive breathlessness, palpitation and cough productive of whitish frothy sputum. There was associated PND, no history of facial or leg swelling.[nigjcardiol.org]
Pulsatile Liver
  • At the other extreme, patients with isolated RVEMF with severe tricuspid regurgitation (TR) present with features of chronic right heart failure with markedly elevated JVP and expansile large 'v' waves, pulsatile liver, hepatomegally, ascites, edema,[nigjcardiol.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]
  • Heart Disease. 5th ed. Philadelphia, Pa:. WB Saunders and Co;1997:1433-4. Zabsonre P, Renambot J, Adoh-Adoh M, et al.[wikidoc.org]
  • In endemic areas of Africa endomyocardial fibrosis is as common a cause of heart failure as rheumatic heart disease, accounting for up to 20% of cases of heart failure and imposes a considerable burden to the communities and the health systems.[globalcardiologyscienceandpractice.com]
  • In the chronic phase, biventricular involvement is the most common presentation, followed by isolated right-sided heart disease. Marked ascites out of proportion to peripheral edema usually develops as a typical feature of EMF.[ncbi.nlm.nih.gov]
S4 Gallop
  • In addition, findings of pulmonary hypertension and S3, S4 gallop are also frequently present. Evaluation Electrocardiogram In advanced stages of EMF, low-voltage QRS complexes and nonspecific ST and T wave abnormalities are observed.[statpearls.com]
Breast Atrophy
  • Physical examination was remarkable for 'egg on stick appearance', central cyanosis, generalized hyper pigmentation, breast atrophy, parotid fullness, and bilateral pitting leg edema.[nigjcardiol.org]
Neglect
  • KEYWORDS: Endomyocarial fibrosis; Heart failure; Neglected diseases; Restrictive cardiomyopathy[ncbi.nlm.nih.gov]
  • Endomyocardial fibrosis (EMF) is a neglected tropical cardiomyopathy of unknown etiology and pathogenesis that is common in certain tropical areas of Africa, Asia, and South America. It affects predominantly children and young adults.[ncbi.nlm.nih.gov]
  • Endomyocardial fibrosis (EMF) is a neglected restrictive cardiomyopathy of unknown aetiology and unclear natural history, which causes premature deaths in endemic areas.[ncbi.nlm.nih.gov]
  • Endomyocardial fibrosis is a neglected research field. It needs more attention from biomedical researchers.[ncbi.nlm.nih.gov]
  • Endomyocardial fibrosis (EMF) is a neglected tropical disease that affects millions of people worldwide. EMF is the most common cause of restrictive cardiomyopathy, caused by deposition of fibrous tissue on endocardial surfaces.[ncbi.nlm.nih.gov]

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]
T Wave Inversion
  • An electrocardiogram revealed symmetrical T-wave inversion, which is a characteristic manifestation of apical hypertrophy.[ncbi.nlm.nih.gov]
  • ECG showed first-degree heart block with ST depression and symmetrical T-wave inversions in the chest leads.[ncbi.nlm.nih.gov]
  • A routine electrocardiogram (ECG) showed deep inferolateral T-wave inversion ( figure 1 ). Figure 2. A. Maximal diastole – minimal left ventricular (LV) pressure. B.[bjcardio.co.uk]
  • 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]
  • The odd finding is a uniform ST segment depression and T wave inversion more evident in the lateral chest leads, similar to apical hypertrophic cardiomyopathy (HCM) and non- ST elevation acute coronary syndromes Not uncommonly, nonspecific ST T wave abnormalities[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]
  • fibrosis are evident, Endomyocardial Fibrosis with Ventricular Thrombosis: Gross, fixed tissue, horizontal section of ventricles showing thrombus filling both.Left ventricle endocardial fibrosis and focal myocardial fibrosis.[wikidoc.org]
  • 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]
  • Diagnosis: “Endo myocardial fibrosis.” The correspondent author’s postmortem report External appearances: The body was that of an emaciated young man who was very pale. The ankles pitted on pressure.[scitcentral.com]
Prominent A-Wave
  • In this case, the only abnormal clinical finding may be a giant or prominent 'a' wave in the jugular venous pulse.[nigjcardiol.org]

Treatment

  • Outcome was favorable under treatment with prednisone and azathioprine.This case illustrates a favorable outcome of endomyocardial fibrosis during pregnancy.[ncbi.nlm.nih.gov]
  • Specific treatment, apart from surgery, remains suboptimal. We report a case of advanced, biventricular EMF complicated by right ventricular outflow tract aneurysms.[ncbi.nlm.nih.gov]
  • The treatment of left ventricular endomyocardial fibrosis with associated mitral valve disease by endocardial resection and mitral valve replacement is a good surgical option that provides good long-term results.[ncbi.nlm.nih.gov]
  • Moreover, the etiology of the disease is unknown, it has no specific treatment, and it carries a poor prognosis.[ncbi.nlm.nih.gov]
  • We discuss imaging and surgical techniques that enable the diagnosis and treatment of endomyocardial fibrosis.[ncbi.nlm.nih.gov]

Prognosis

  • Endomyocardial fibrosis carries a poor prognosis, due to the late presentation of patients.[ncbi.nlm.nih.gov]
  • CONCLUSIONS: Our study provides evidence that LGE-CMR is useful in the diagnosis and prognosis of EMF through quantification of the typical pattern of FT deposition.[ncbi.nlm.nih.gov]
  • Moreover, usual presentation of this condition is in advanced stage with poor prognosis. Ventricular endocardial fibrosis with organised thrombus is the hall mark of this entity. Presence of associated cardiac mass poses a diagnostic challenge.[ncbi.nlm.nih.gov]
  • Endomyocardial fibrosis is an uncommon cause of congestive cardiac failure characterized by severe diffuse thickening of the endocardium, and the overall long-term prognosis of this disease is generally considered poor.[ncbi.nlm.nih.gov]
  • Moreover, the etiology of the disease is unknown, it has no specific treatment, and it carries a poor prognosis.[ncbi.nlm.nih.gov]

Etiology

  • Endomyocardial fibrosis (EMF) is a rare progressive restrictive cardiomyopathy of unknown etiology that mainly presents with heart failure.[ncbi.nlm.nih.gov]
  • Endomyocardial fibrosis (EMF) is a neglected tropical cardiomyopathy of unknown etiology and pathogenesis that is common in certain tropical areas of Africa, Asia, and South America. It affects predominantly children and young adults.[ncbi.nlm.nih.gov]
  • Moreover, the etiology of the disease is unknown, it has no specific treatment, and it carries a poor prognosis.[ncbi.nlm.nih.gov]
  • Abstract Tropical endomyocardial fibrosis (TEMF), a restrictive cardiomyopathy of unclear etiology, is an endemic disease in equatorial Africa, South America and India.[ncbi.nlm.nih.gov]
  • Endomyocardial fibrosis is a restrictive cardiomyopathy, of unknown etiology, which occurs most commonly in tropical and subtropical areas.[ncbi.nlm.nih.gov]

Epidemiology

  • 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]
  • 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]
  • Part II: An epidemiologic, clinical, and pathologic study. Am Heart J 1968; 75: 107–124. Connor DH, Somers K, Hutt MS, et al. Endomyocardial fibrosis in Uganda (Davies’ disease). Part I: An epidemiologic, clinical, and pathologic study.[jrcd.eu]
  • Advances have, however, been made in establishing the epidemiology and improving clinical diagnosis and management, through modern medical therapy and improved surgical techniques.[ncbi.nlm.nih.gov]
  • 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]
Sex distribution
Age distribution

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]
  • Research is still required to define clinical, biological and echocardiographic markers of early stages of EMF, so that advances in the knowledge of its pathogenesis and pathophysiology can be made.[ncbi.nlm.nih.gov]
  • 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]
  • […] including hypersensitivity myocarditis [14], parasitic infections [12,13], eosinophilic leukemia, sarcoma, carcinoma, and lymphoma [15], GM-CSF administration [16], and prolonged drug-induced eosinophilia Several infections have been implicated in the pathophysiology[slideshare.net]
  • However, there is need to fully understand the pathogenesis and pathophysiology of EMF in order to improve the management and outcome of the affected patients.[link.springer.com]

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]
  • Better nutrition would protect against Magnesium deficiency and prevent the absorption of Cerium in the patients from Alleppey, compared to those from Trivandrum. Copyright 2016. Published by Elsevier B.V.[ncbi.nlm.nih.gov]
  • This will hopefully determine preventive measures and avoid the burden of this debilitating condition in this continent.[ncbi.nlm.nih.gov]
  • Increased awareness among health workers and policy makers is the need of the hour for the unhindered development of efficient preventive and therapeutic strategies. 2016 American Heart Association, Inc.[ncbi.nlm.nih.gov]
  • While RHD is unarguably the most preventable of all cardiac diseases, resulting from untreated or undertreated group A streptococcal infections, EMF’s etiology remains unclear.[journals.ac.za]

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