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Endocardial Fibroelastosis

Endomyocardial Fibroelastosis


Presentation

  • Three cases of endocardial fibroelastosis with vectorcardiographic findings suggestive of an anterior myocardial infarct are presented.[ncbi.nlm.nih.gov]
  • The echocardiographic diagnosis made at presentation was critical aortic stenosis in all cases.[ncbi.nlm.nih.gov]
  • We report a case of dilated cardiomyopathy presented in a premature infant of 27 weeks' gestational age. A prenatal sonography revealed left ventricular enlargement. The infant presented with respiratory distress and heart failure soon after birth.[ncbi.nlm.nih.gov]
  • Endocardial fibroelastosis is a cardiomyopathy not commonly seen in the present age.[ncbi.nlm.nih.gov]
  • Furthermore, this case presented striking histopathological differences from scleroderma neonatorum and infantile subcutaneous fat necrosis in many respects.[ncbi.nlm.nih.gov]
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]
  • Clinical presentation varies from asymptomatic patients to those with congestive heart failure secondary to significant valvular regurgitation and low right ventricular output.[ncbi.nlm.nih.gov]
  • A 10-year-old boy with congestive heart failure died in five months in spite of comprehensive medical treatment. Autopsy showed patchy areas of endocardial fibroelastosis of the left ventricle.[ncbi.nlm.nih.gov]
  • Intrauterine congestive heart failure was thought to have been the probable cause of hydrops in eight of the 10 infants with endocardial fibroelastosis.[ncbi.nlm.nih.gov]
  • We report a case of an 18-month-old male, born to a woman with third trimester febrile illness, who had a history of congestive heart failure and respiratory distress, cardiomegaly, and electrocardiographic (ECG) findings suggestive of cardiomyopathy[ncbi.nlm.nih.gov]
Collapse
  • A four-month-old female with no previous medical history suddenly collapsed and failed to recover despite 2h of resuscitation.[ncbi.nlm.nih.gov]
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]
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]
  • RATIONALE: Endocardial fibroelastosis (EFE) is a unique form of fibrosis, which forms a de novo subendocardial tissue layer encapsulating the myocardium and stunting its growth, and which is typically associated with congenital heart diseases of heterogeneous[ncbi.nlm.nih.gov]
  • Endocardial fibroelastosis is a congenital heart disease known to cause congestive heart failure in early infancy. We report a case using new modalities for early prenatal diagnosis of endocardial fibroelastosis causing fetal heart failure.[ncbi.nlm.nih.gov]
  • A comprehensive and exhaustive reference of fundamental and clinical aspects of heart disease in infancy and childhood.[books.google.de]
  • Both Webcasts are part of this year's "Frontiers in Diagnosis & Management of Congenital Heart Disease," taking place at Children's Hospital Boston.[orlive.com]
Gallop Rhythm
  • rhythm, persistent jugular venous distention, systemic hypotension, peripheral vascular disease Laboratory findings Hyponatremia, persistently elevated B-type natriuretic peptide and A-type natriuretic peptide levels, elevated norepinephrine and renin[clevelandclinicmeded.com]
Skin Lesion
  • Although the skin lesion is similar to that of sclerema neonatorum presented by HUGHES et al., systemic fibrosis in the visceral organs has not been reported in the literature with respect of this disease.[ncbi.nlm.nih.gov]
Communicating Hydrocephalus
  • We report on two unrelated male infants with similar findings of communicating hydrocephalus, endocardial fibroelastosis (EFE) and congenital cataracts, who died at 4 months of age.[ncbi.nlm.nih.gov]
Cesarean Section
  • In order to minimize the risk of recurrence in further pregnancies, elective cesarean section may be considered.[ncbi.nlm.nih.gov]

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 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]
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]
Hepatic Necrosis
  • Hepatic necrosis was notable in 3 patients (patients 1, 3, and 21 in Table 2 ), while cerebral infarction (patient 7, Table 2 ) and renal infarction (patient 25, Table 2 ) occurred in 1 child each.[dx.doi.org]

Treatment

  • The prognosis mainly depends on the early and accurate diagnosis and treatment. However, without a typical and specific clinical manifestation in early stage, ALCAPA has a higher rate of false initial diagnosis.[ncbi.nlm.nih.gov]
  • Cardiovascular evaluation should be performed during the course of ritodrine treatment, and no patient should be discharged if she does not have normal cardiovascular function.[ncbi.nlm.nih.gov]
  • Surgical treatment of mitral insufficiency and aortic valve stenosis associated with endocardial fibroelastosis has not been well documented. We treated 2 infants with this complex lesion: in one case it was possible to repair the mitral valve.[ncbi.nlm.nih.gov]
  • A 10-year-old boy with congestive heart failure died in five months in spite of comprehensive medical treatment. Autopsy showed patchy areas of endocardial fibroelastosis of the left ventricle.[ncbi.nlm.nih.gov]
  • The patient improved following treatment with imatinib.[ncbi.nlm.nih.gov]

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]
  • Data were sampled from the clinical symptoms, eletrocardiography, echocardiography, electron beam computed tomography (EBCT), management, and prognosis.[ncbi.nlm.nih.gov]
  • The CVPS of this affected fetus dropped to 6 out of 10 points, which indicated a severe heart condition along with the fetus and predicted an adverse fetal prognosis according to our recommendation system.[ncbi.nlm.nih.gov]
  • Awareness of this condition is essential for prompt recognition and referral to a tertiary cardiac center to enable early surgical intervention and improved prognosis for these children.[ncbi.nlm.nih.gov]
  • EFE is a rare cardiac disorder with poor prognosis and uncertain cause. Primary and secondary forms have been described. Most authors consider that all EFE is secondary--a reactive process set off in the endocardium by stress on the myocardium.[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]
  • The etiology of her cardiomyopathy was unclear. Inhaled prostacyclin therapy resulted in an improvement of pulmonary arterial pressure and allowed us to avoid lung transplantation.[ncbi.nlm.nih.gov]
  • We discuss the association of birth defects in widely separated organ systems in these patients and suggest that this may represent a genetic syndrome; however, a viral etiology cannot entirely be excluded.[ncbi.nlm.nih.gov]
  • These data suggest an etiologic role for viral infection in endocardial fibroelastosis, supporting the hypothesis that endocardial fibroelastosis is a sequela of a viral myocarditis, in particular of that due to mumps virus.[ncbi.nlm.nih.gov]
  • Autoantibody-mediated EFE may be an etiologic factor in cases of fetal and neonatal "idiopathic" dilated cardiomyopathy.[ncbi.nlm.nih.gov]

Epidemiology

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

Pathophysiology

  • We also discuss the pathophysiology of this association and related literature.[ncbi.nlm.nih.gov]
  • The central hypothesis of these studies has been that by identifying the pathophysiological pathways activated by these mutations we can define enzymatic activities that are modified during the disease process and which may be involved in pathways that[ncbi.nlm.nih.gov]
  • Understanding of the pathophysiology of acute myocardial infarcts has matured sufficiently so that now it is possible to consider whether an infarct must evolve to a predetermined size or whether the size could be limited by implementing one or more clinically[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]
  • […] 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]
  • Treatment of CHB Curative treatment of CHB and prophylactic treatment to prevent the recurrence of CHB are two major issues.[arthritis-research.biomedcentral.com]

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