High prevalence of endomyocardial fibrosis in rural. 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. Endomyocardial fibrosis emf, a restrictive cardiomyopathy, is one of the commonest cardiac conditions in uganda. It is also used as a diagnostic tool in some heart diseases. Tropical endomyocardial fibrosis is a common cause of restrictive cardiomyopathy worldwide, but is. Forme fruste of isolated right ventricular endomyocardial. Endomyocardial fibrosis with aortic, mitral, and tricuspid. We report the case of a young african woman with a history of right ventricular failure.
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. It results in endocardial rigidity, atrioventricular valve incompetence secondary to papillary. 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 is a neglected tropical disease of unknown etiology and poor prognosis. Endomyocardial fibrosis emf is a common cause of primary restrictive cardiomyopathy rcm in the tropics. Endomyocardial fibrosis emf is a specific clinicopathological entity. The right ventricle was extremely small and restricted. The data on emf associated with pregnancy are scarce.
To the present time no treatment has been effective in controlling the evolution of the process. Endomyocardial biopsy emb is routinely performed for cardiac transplant monitoring. Download article pdf view full text html machine readable. 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. Between 1980 and 1998, we studied 10 women aged 27 to 58 years with endomyocardial fibrosis confirmed by surgery andor endomyocardial biopsy. 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 radiology reference article. This article is from journal of medical case reports, volume 8.
The hallmark feature of endomyocardial fibrosis rcm is. Endomyocardial fibrosis and myocardial infarction leading to diastolic and systolic dysfunction requiring transplantation. The nosology of emf coincides with some related disorders. This case illustrates how subclinical myocardial inflammation due to chronic hypereosinophilia could cause endomyocardial fibrosis, systolic dysfunction, and thrombus. It is a condition with high morbidity and mortality, for which no effective therapy is available. 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. 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. Endomyocardial fibrosis emf, a disease encountered in tropical areas, characteristically in uganda, produces progressive endocardial restriction, heart failure, and ultimately death. Endomyocardial fibrosis emf is an idiopathic disorder characterized by the development of restrictive cardiomyopathy.
The unique nature of this condition allowed a diagnosis prior to surgery in the second case. Endomyocardial fibrosis is a form of endemic restrictive cardiomyopathy that affects mainly children and adolescents. For full access to this pdf, sign in to an existing account, or purchase an annual subscription. Endomyocardial fibrosis ef is characterized by marked endocardial thickening of the apex and subvalvular apparatus of one or both ventricles. We recently saw a patient with endomyocardial fibrosis of the left ventricle, with aortic, mitral, and tricuspid valve involvement. The surgical approach entailed endocardectomy and a bidirectional cavopulmonary shunt to improve weaning off bypass and postoperative recovery, both of which. A myocardial biopsy of the right ventricle confirmed the presence of endomyocardial fibrosis.
The majority of people who suffer high morbidity and mortality are children and young females of the poor and deprived. Although endomyocardial fibrosis is clearly an idiopathic restrictive cardiomyopathy typically. Usefulness of cardiac mri in the early diagnosis of endomyocardial. Endomyocardial fibrosis nord national organization for. Right ventricular endomyocardial biopsy showing a prominent layer of fibrin on. One form of rcm is associated with endomyocardial fibrosis. Cardiac magnetic resonance in tropical endomyocardial fibrosis. Epidemiology it usually occurs in tropical and subtropical regions of the world.
Endomyocardial fibrosis syndrome in uganda the lancet. 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. Endomyocardial fibrosis symptoms, diagnosis, treatments and. 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. A prominent layer of fibrin was noted on the endocardial surface with interstitial fibrosis involving the underlying myocardium. Endomyocardial fibrosis emf is a rare disease in north america but common in the tropical and subtropical regions of the developing world. Endomyocardial fibrosis emf is a form of restrictive cardiomyopathy mainly present in tropical areas. Here we report a rare case of right ventricular endomyocardial fibrosis. Endomyocardial fibrosis and myocardial infarction leading to. 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.
General discussion endomyocardial fibrosis emf is a progressive disease of unknown origin idiopathic that may seriously affect the heart. The clinical manifestations are largely related to the consequences of restrictive ventricular filling, including left and right sided heart failure. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. Right ventricular endomyocardial fibrosis a case report. The case of a young girl with emf and unrelenting congestive heart failure is presented.
Endomyocardial fibrosis and apical thrombus in patient with. Endomyocardial fibrosis cleveland clinic journal of medicine. Hypereosinophilic syndrome is a disease characterized by a persistently elevated eosinophil count. Isolated severe right ventricle endomyocardial fibrosis in. Following initiation of conventional treatment, the patient remained stable from the cardiovascular standpoint. Combined endocardiectomy and bidirectional glenn shunt for. Nov 24, 2014 in general, the response to medical therapy is unproven due to lack of welldesigned studies. Endomyocardial fibrosis symptoms, diagnosis, treatments. The diagnosis of endomyocardial fibrosis associated with this bivalvular endocarditis was made on the basis of this typical mri finding. 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. This lady had endomyocardial fibrosis emfa nearly missed diagnosis had it not been for cmr fb moco lge.
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. Number of publications in medline between 1950 and 2006 with either endomyocardial fibrosis or endomyocardial sclerosis in the title. Endomyocardial fibrosis and restrictive cardiomyopathy. Tropical endomyocardial fibrosis in india was a common medical problem in the coastal districts of south india, especially the kerala state. Endomyocardial fibrosis and myocardial infarction leading.
On computed tomography, massive myocardial calcification was seen, mainly at the left ventricle and interventricular septum fig. Tropical endomyocardial fibrosis circulation aha journals. The clinical and autopsy studies have shown left and right ventricular apical fibrosis, with varying degree of atrioventricular valve regurgitation. Visualization of endomyocardial fibrosis by delayed.
Distinguishing between the two is important, as management and prognosis differs. For full access to this pdf, sign in to an existing account, or purchase an annual. 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. In south america, patients with emf have come from brazil and columbia rather than peru or ecuador. 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. Download fulltext pdf download fulltext pdf electrocardiogram in endomyocardial fibrosis article pdf available in indian pacing and electrophysiology journal 115. Emf closely resembles late stage loefflers endocarditis le. 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. Idiopathic neglected cardiovascular disease restrictive cardiomyopathy tropical disease. For claims with a date of service on or after october 1, 2015, use an. If you have problems viewing pdf files, download the latest version of adobe reader. Endomyocardial fibrosis and apical thrombus in patient. Endomyocardial fibrosis emf is a form of restrictive cardiomyopathy of unknown etiology. Cardiac magnetic resonance imaging and eosinophilic.
Endomyocardial fibrosis and unileaflet mitral valve. Surgical therapy by endocardial decortication seems to be beneficial for many patients with advanced disease who are in functional. Endomyocardial fibrosis definition of endomyocardial. 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.
The patient resided in madrid and had never been to africa. 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. Tropical endomyocardial fibrosis emf is a neglected disease of poverty that afflicts rural populations in tropical lowincome countries, with some certain highprevalence areas. Postoperatively, the patient developed refractive congestive heart failure, which progressed to cardiogenic shock and death. Usefulness of echocardiography and doppler echocardiography. 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. In the absence of nearly any functional rv cavity, heart transplantation was the only distant curative option left for the patient. 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. Emf diagnosis is usually based on the detection of structural and functional cardiac abnormalities by cardiac imaging. 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. Pdf endomyocardial fibrosis emf is a neglected idiopathic disorder, predominant in tropical and subtropical. 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.
In sporadic cases, blood eosinophilia is a common feature. 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. Diagnosis and management of endomyocardial fibrosis. This pattern was considered diagnostic of endomyocardial fibrosis and surgery was advised. This results in constriction of the heart cavity and may involve the heart valves and other structures.
Endomyocardial fibrosis emf is a fascinating disease entity of unknown etiology. Pitfalls in diagnosis and clinical, echocardiographic, and. It occurs sporadically throughout the world but is endemic in parts of africa, india and south america. Forme fruste of isolated right ventricular endomyocardial fibrosis. Endomyocardial fibrosis with massive left ventricular calcification. Furthermore, malaria has never been described as being associated with myocardial or endocardial damage. For language access assistance, contact the ncats public information officer. Clinicopathologic features in cats are currently poorly defined.
The patient underwent left ventricle endocardiectomy, aortic and. Comprehensive assessment of endomyocardial fibrosis with. The case we report, shows a successful treatment of right ventricle endomyocardial fibrosis. Endomyocardial fibrosis emf is a progressive type of restrictive cardiomyopathy. It affects inflow portion of right andor left ventricle and apex.