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Endocardial Fibroelastosis
Endocardial Fibroelastoses

Presentation

Three cases of endocardial fibroelastosis with vectorcardiographic findings suggestive of an anterior myocardial infarct are presented. [ncbi.nlm.nih.gov]

Entire Body System

  • Congestive Heart Failure

    Endocardial fibroelastosis is an important cause of congestive heart failure and death in infancy and early childhood. When present, it is commonly associated with non immune hydrops fetalis. [ncbi.nlm.nih.gov]

Gastrointestinal

  • Failure to Thrive

    Symptoms - Endocardial fibroelastosis Breathlessness Cough Wheezing Feeding difficulty Excessive sweating Failure to thrive Recurrent chest infections (20%) Causes - Endocardial fibroelastosis EFE has two distinct genetic forms, each having a different [checkorphan.org]

Cardiovascular

  • Heart Disease

    The EFE + heart disease group had both fewer B cells (0.18 vs. 0.44 cells/mm(2); p = 0.08) and T cells (0.29 vs. 0.80 cells/mm(2); p = 0.04) than the control group. [ncbi.nlm.nih.gov]

    Now, in full color, this leading reference offers you everything you need to treat and manage pediatric heart conditions. A comprehensive and exhaustive reference of fundamental and clinical aspects of heart disease in infancy and childhood. [books.google.de]

    It presents as unexplained heart failure in infants and children. The disease can be primary or secondary to various congenital heart diseases, most notably hypoplastic left heart syndrome, aortic stenosis, or atresia. [chdbabies.com]

  • Gallop Rhythm

    Examination should focus on whether the heart is palpably dilated, the presence of murmurs, and additional heart sounds (gallops). [clevelandclinicmeded.com]

Workup

Today, if a patient presented with myocarditis ( pdf on endocarditis and myocarditis), a complete workup would be done. First, the doctor would do a physical exam, then an electrocardiogram and blood work. [theancestorfiles.blogspot.com]

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

    Observations that favor a viral etiology include a clinical presentation similar to that of chronic myocarditis, findings of myocarditis or myocardial fibrosis in affected patients, a higher incidence following epidemics of coxsackievirus B infection, [chdbabies.com]

    The combination of myocyte injury and necrosis associated with myocardial fibrosis results in impaired mechanical function. [clevelandclinicmeded.com]

  • Lymphocytic Infiltrate

    Additionally, there was a mixture of myocardial fibers with a marked "wavy" appearance and a scattering of mild interstitial lymphocytic infiltration. [ncbi.nlm.nih.gov]

Treatment

The patient improved following treatment with imatinib. [ncbi.nlm.nih.gov]

Treatment - Endocardial fibroelastosis The principles are the same as treatment of chronic cardiac failure. * Any acute exacerbations are often precipitated by respiratory infections. * Early and prolonged treatment with digoxin is suggested. * Therapy [checkorphan.org]

This non-surgical treatment restores effective pulmonary valve function and potentially prolongs the functional life of regurgitant and/or stenotic right ventricle to pulmonary artery conduits. [orlive.com]

Prognosis

Middle-term prognosis of isolated autoantibody-associated EFE may be better than previously reported, although the longterm prognosis remains unknown. We hypothesize that a fetal insult can lead to DCM. [ncbi.nlm.nih.gov]

Etiology

We propose that the presence of these particles in this case in association with the other morphological alterations in support for the viral etiology of EFE. [ncbi.nlm.nih.gov]

It has also been suggested to be a nonspecific response to chronic myocardial dysfunction. 4) In our case, we could not find any etiology for the EFE. [synapse.koreamed.org]

Theories of etiology : hypoxia,congenital, ...etc. B. Gross Findings : Diffuse or patchy fibroelastic thickening of the endocardium,mostly on the left,occasionly with overlying mural thrombi. Cardiomegaly due to LV hypertrophy and or dilation. C. [pathology.class.kmu.edu.tw]

Epidemiology

OBJECTIVES: To analyze the epidemiologic, clinical and anatomopathological aspects of primary EFE. [scielo.br]

An epidemiologic assessment of primary endocardial fibroelastosis. Am J Cardiol. 1966 Dec. 18(6):859-66. [Medline]. Nield LE, Silverman ED, Smallhorn JF, et al. [emedicine.medscape.com]

Recent advances in the epidemiology, diagnosis and treatment of endomyocardial fibrosis in Africa. Heart. 2013;99:1481-7. Mavrogeni S, Sfikakis PP, Gialafos E, et al. [escardio.org]

Pathophysiology

We also discuss the pathophysiology of this association and related literature. [ncbi.nlm.nih.gov]

Grasp the full scope of the pathophysiology of CHD with well-organized, expert guidance. Quickly search the contents online, download images in JPG or PPT format, and view 70 echocardiogram videos at expertconsult.com. [books.google.de]

Pathophysiology Dilated cardiomyopathy represents the final common morphologic outcome of various biologic insults. The combination of myocyte injury and necrosis associated with myocardial fibrosis results in impaired mechanical function. [clevelandclinicmeded.com]

Prevention

The contracted form of primary fibroelastosis in the fetus must have followed an insult that prevented any further increase in the size of the left ventricular cavity. [ncbi.nlm.nih.gov]

There is also some evidence that viral infection may play a role as a cause or trigger Prevention - Endocardial fibroelastosis Not supplied. [checkorphan.org]

Take good care of your teeth and gums to prevent infections. Make regular visits to your dentist. Take antibiotics before you have any medical work that may cause bleeding, like dental work and most surgeries. [webmd.com]

Treatment of CHB Curative treatment of CHB and prophylactic treatment to prevent the recurrence of CHB are two major issues. [arthritis-research.biomedcentral.com]

[…] that the enlarged heart and heart failure was caused by a viral infection or a viral infection in conjunction with a congenital heart defect, the vaccine development was too late to save her life, but Hilleman's "measles vaccine alone is estimated to prevent [theancestorfiles.blogspot.com]

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