Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. Endomyocardial fibrosis is a cause of restrictive cardiomyopathy endemic to tropical regions which in nonendemic countries remains a diagnostic challenge with the risk of late treatment beginning and uncertain outcome. Endomyocardial fibrosis ef is characterized by marked endocardial thickening of the apex and subvalvular apparatus of one or both ventricles. Nov 24, 2014 endomyocardial fibrosis emf is an idiopathic disorder of the tropical and subtropical regions of the world that is characterized by the development of restrictive cardiomyopathy. This results in constriction of the heart cavity and may involve the heart valves and other structures. Isolated severe right ventricle endomyocardial fibrosis in. Although endomyocardial fibrosis is clearly an idiopathic restrictive cardiomyopathy typically. The unique nature of this condition allowed a diagnosis prior to surgery in the second case. Endomyocardial fibrosis symptoms, diagnosis, treatments and. Endomyocardial fibrosis and restrictive cardiomyopathy.
In sporadic cases, blood eosinophilia is a common feature. Endomyocardial biopsy emb is an invasive procedure used routinely to obtain small samples of heart muscle, primarily for detecting rejection of a donor heart following heart transplantation. Endomyocardial fibrosis with aortic, mitral, and tricuspid. Endomyocardial fibrosis definition of endomyocardial. The hallmark feature of endomyocardial fibrosis rcm is. Visualization of endomyocardial fibrosis by delayed. Diagnosis and management of endomyocardial fibrosis. To the present time no treatment has been effective in controlling the evolution of the process. Hypereosinophilic syndrome is a disease characterized by a persistently elevated eosinophil count. It affects inflow portion of right andor left ventricle and apex. Emf diagnosis is usually based on the detection of structural and functional cardiac abnormalities by cardiac imaging. Idiopathic neglected cardiovascular disease restrictive cardiomyopathy tropical disease.
Endomyocardial fibrosis emf is a fascinating disease entity of unknown etiology. Endomyocardial fibrosis emf, a restrictive cardiomyopathy, is one of the commonest cardiac conditions in uganda. A myocardial biopsy of the right ventricle confirmed the presence of endomyocardial fibrosis. Pdf endomyocardial fibrosis emf is a neglected idiopathic disorder, predominant in tropical and subtropical. For full access to this pdf, sign in to an existing account, or purchase an annual subscription. Endomyocardial fibrosis emf is a specific clinicopathological entity. Endomyocardial fibrosis symptoms, diagnosis, treatments. Download article pdf view full text html machine readable. Introduction endomyocardial fibrosis emf is a disease that is characterized by fibrosis of the apical endocardium of the right ventricle rv, left ventricle lv, or both. The right ventricle was extremely small and restricted. Endomyocardial fibrosis emf is a common cause of primary restrictive cardiomyopathy rcm in the tropics. Endomyocardial fibrosis emf affects approximately 12 million persons worldwide and is an important cause of restrictive cardiomyopathy in the developing world, with the highest prevalence reported in subsaharan africa, south asia, and south america. On computed tomography, massive myocardial calcification was seen, mainly at the left ventricle and interventricular septum fig.
Because most patients with endomyocardial fibrosis emf present long after any possible period of early active myocarditis may have existed, little, if any, role exists for immunosuppressive therapy as is used in some patients with loeffler disease. This pattern was considered diagnostic of endomyocardial fibrosis and surgery was advised. Nov 24, 2014 in general, the response to medical therapy is unproven due to lack of welldesigned studies. The patient resided in madrid and had never been to africa. Usefulness of cardiac mri in the early diagnosis of endomyocardial. Jul 17, 2012 endomyocardial fibrosis is a form of endemic restrictive cardiomyopathy that affects mainly children and adolescents, and is geographically restricted to some poor areas of africa, latin america and asia. Endomyocardial fibrosis emf, a disease encountered in tropical areas, characteristically in uganda, produces progressive endocardial restriction, heart failure, and ultimately death. We want to share our experience of nearly 20 years to demonstrate the usefulness of echocardiography and echodoppler in the diagnosis and treatment of endomyocardial fibrosis. Endomyocardial fibrosis and unileaflet mitral valve. Cardiac magnetic resonance in tropical endomyocardial fibrosis.
The patient underwent left ventricle endocardiectomy, aortic and. For full access to this pdf, sign in to an existing account, or purchase an annual. Between 1980 and 1998, we studied 10 women aged 27 to 58 years with endomyocardial fibrosis confirmed by surgery andor endomyocardial biopsy. Tropical endomyocardial fibrosis emf is a neglected disease of poverty that afflicts rural populations in tropical lowincome countries, with some certain highprevalence areas.
The patient underwent endomyocardial stripping, mitral valve replacement, and tricuspid ring valvuloplasty. Our goal was to demonstrate the usefulness of echocardiography and cardiac doppler echocardiography echodoppler in the diagnosis of endomyocardial fibrosis, an unusual restrictive cardiomyopathy in argentina. Abstracttwo cases of endomyocardial fibrosis seen in the state of ohio are described and related to the incidence and prevalence of endomyocardial fibrosis throughout the world. The diagnosis of endomyocardial fibrosis associated with this bivalvular endocarditis was made on the basis of this typical mri finding.
Furthermore, malaria has never been described as being associated with myocardial or endocardial damage. Number of publications in medline between 1950 and 2006 with either endomyocardial fibrosis or endomyocardial sclerosis in the title. It is also used as a diagnostic tool in some heart diseases. Endomyocardial fibrosis emf is a progressive type of restrictive cardiomyopathy. Endomyocardial fibrosis syndrome in uganda the lancet. In south america, patients with emf have come from brazil and columbia rather than peru or ecuador. We report the case of a young african woman with a history of right ventricular failure.
Endomyocardial fibrosis is a form of endemic restrictive cardiomyopathy that affects mainly children and adolescents, and is geographically restricted to some poor areas of africa, latin america and asia. The clinical manifestations are largely related to the consequences of restrictive ventricular filling, including left and right sided heart failure. We recently saw a patient with endomyocardial fibrosis of the left ventricle, with aortic, mitral, and tricuspid valve involvement. Endomyocardial fibrosis emf is a neglected idiopathic disorder, predominant in tropical and subtropical regions of the developing world. The majority of people who suffer high morbidity and mortality are children and young females of the poor and deprived. The surgical approach entailed endocardectomy and a bidirectional cavopulmonary shunt to improve weaning off bypass and postoperative recovery, both of which. This article is from journal of medical case reports, volume 8.
Image studies suggested endomyocardial fibrosis affecting only the right side of the heart. Endomyocardial fibrosis and myocardial infarction leading to diastolic and systolic dysfunction requiring transplantation. Endomyocardial fibrosis is a neglected tropical disease of unknown etiology and poor prognosis. Endomyocardial fibrosis with massive left ventricular calcification. General discussion endomyocardial fibrosis emf is a progressive disease of unknown origin idiopathic that may seriously affect the heart. Apr 02, 2015 introduction endomyocardial fibrosis emf is a disease that is characterized by fibrosis of the apical endocardium of the right ventricle rv, left ventricle lv, or both. The data on emf associated with pregnancy are scarce. High prevalence of endomyocardial fibrosis in rural. Endomyocardial fibrosis emf is a form of restrictive cardiomyopathy of unknown etiology, characterized by endocardial fibrosis of the apices and inflow tracts of the right ventricle, left ventricle or both. It is a condition with high morbidity and mortality, for which no effective therapy is available. The case of a young girl with emf and unrelenting congestive heart failure is presented. Emf closely resembles late stage loefflers endocarditis le. If you have problems viewing pdf files, download the latest version of adobe reader.
Endomyocardial fibrosis emf is a form of restrictive cardiomyopathy of unknown etiology. Endomyocardial fibrosis emf is a restrictive cardiomyopathy of unknown etiology prevalent in tropical regions affecting the inflow tract and apex of one or both ventricles, which show fibrous. This case illustrates how subclinical myocardial inflammation due to chronic hypereosinophilia could cause endomyocardial fibrosis, systolic dysfunction, and thrombus. Endomyocardial fibrosis emf is a form of restrictive cardiomyopathy mainly present in tropical areas. This lady had endomyocardial fibrosis emfa nearly missed diagnosis had it not been for cmr fb moco lge. Endomyocardial fibrosis emf is a rare disease in north america but common in the tropical and subtropical regions of the developing world. Epidemiology it usually occurs in tropical and subtropical regions of the world. It results in endocardial rigidity, atrioventricular valve incompetence secondary to papillary. Endomyocardial fibrosis emf is an idiopathic disorder characterized by the development of restrictive cardiomyopathy. Right ventricular endomyocardial biopsy showing a prominent layer of fibrin on. Following initiation of conventional treatment, the patient remained stable from the cardiovascular standpoint. A prominent layer of fibrin was noted on the endocardial surface with interstitial fibrosis involving the underlying myocardium. Endomyocardial fibrosis and myocardial infarction leading to. The hallmark feature of endomyocardial fibrosis rcm is severe endomyocardial scar that may diffusely involve the left ventricle, or appear as a distinct lesion bridging the ventricular septum and left ventricular free wall.
Clinicopathologic features in cats are currently poorly defined. Endomyocardial fibrosis is a form of endemic restrictive cardiomyopathy that affects mainly children and adolescents. Andrea romero valero, francisco javier coves orts, tania muci, pedro morillas blasco, endomyocardial fibrosis and apical thrombus in patient with hypereosinophilia, european heart journal, volume 40. For language access assistance, contact the ncats public information officer. The nosology of emf coincides with some related disorders. Endomyocardial fibrosis is a form of endemic restrictive cardiomyopathy that affects mainly children and adolescents, and is geographically restricted to some poor areas of africa, latin america. Here we report a rare case of right ventricular endomyocardial fibrosis.
Download fulltext pdf download fulltext pdf electrocardiogram in endomyocardial fibrosis article pdf available in indian pacing and electrophysiology journal 115. Endomyocardial fibrosis emf is a disease of rural poverty that is characterized by fibrosis of the apical endocardium of the right ventricle rv, left ventricle lv, or both. In the absence of nearly any functional rv cavity, heart transplantation was the only distant curative option left for the patient. Tropical endomyocardial fibrosis is a common cause of restrictive cardiomyopathy worldwide, but is. Endomyocardial fibrosis and apical thrombus in patient with. Usefulness of echocardiography and doppler echocardiography. On the basis of classical clinical and echocardiographic findings, diagnosis of isolated rv endomyocardial fibrosis was confirmed, and the child was managed with vigorous decongestive treatment and anticoagulation. Endomyocardial biopsy emb is routinely performed for cardiac transplant monitoring. Endomyocardial fibrosis radiology reference article. Endomyocardial fibrosis and myocardial infarction leading. Its most obvious feature is a gross change in the makeup of the lining of the heart cavities the endocardium of one or both of the lower chambers of the heart the ventricles leading to the replacement of normal cells with fibrous tissue fibrosis. Right ventricular endomyocardial fibrosis a case report. Distinguishing between the two is important, as management and prognosis differs. Tropical emf is characterized by the deposition of fibrous tissue in the endomyocardium, leading to restrictive physiology.
Pdf tropical endomyocardial fibrosis emf is a neglected disease. Tropical endomyocardial fibrosis circulation aha journals. Pitfalls in diagnosis and clinical, echocardiographic, and. Its essential features are the formation of fibrous tissue on the endocardium and to a lesser extent in the myocardium of the inflow tract and apex of one or both ventricles. Endomyocardial fibrosis cleveland clinic journal of medicine.
It occurs sporadically throughout the world but is endemic in parts of africa, india and south america. Surgical therapy by endocardial decortication seems to be beneficial for many patients with advanced disease who are in functional. Cardiac magnetic resonance imaging and eosinophilic. Tropical endomyocardial fibrosis in india was a common medical problem in the coastal districts of south india, especially the kerala state. Endomyocardial fibrosis and apical thrombus in patient. Moreover, it can be a useful tool in the diagnostic workup of myocarditis, cardiomyopathies, drug toxicity, unexplained arrhythmias, heart involvement in systemic disease, and to diagnose cardiac masses. Late gadolinium enhancement lge imaging demonstrated the v sign of endomyocardial fibrosis emf at the apex, 1 characterized by a threelayered appearance of dark overlying thrombus, bright fibrotic endomyocardium, and dark underlying myocardium figure 1, arrows. The clinical and autopsy studies have shown left and right ventricular apical fibrosis, with varying degree of atrioventricular valve regurgitation.
Heart disease characterized by progressive fibrosis of the lining of one or both lower heart cavities. Forme fruste of isolated right ventricular endomyocardial fibrosis. One form of rcm is associated with endomyocardial fibrosis. Comprehensive assessment of endomyocardial fibrosis with. Endomyocardial fibrosis nord national organization for. Late gadolinium enhancement lge imaging demonstrated the v sign of endomyocardial fibrosis emf at the apex, 1 characterized by a threelayered appearance of dark overlying thrombus, bright fibrotic endomyocardium. The case we report, shows a successful treatment of right ventricle endomyocardial fibrosis. Endomyocardial fibrosis is a neglected tropical disease of unknown. Endomyocardial fibrosis emf is an idiopathic disorder of the tropical and subtropical regions of the world that is characterized by the development of restrictive cardiomyopathy. Combined endocardiectomy and bidirectional glenn shunt for.