Endomyocardial fibrosis presented by ventricular tachycardia: case report
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Endomyocardial fibrosis (EMF) is a form of restrictive cardiomyopathy that is diagnosed mainly in children and young adults and is geographically found in Africa, Latin America, and Asia. It is a condition with high morbidity and mortality, unknown etiology, and no definitive treatment. Although its main clinical presentation is congestive heart failure with or without related supraventricular arrhythmia like atrial fibrillation, it very rarely presents with ventricular arrhythmias and tachycardias (VA, VT).
We report a case of right ventricular (RV) EMF presented with recurrent attacks of hemodynamically unstable VT that required direct current (DC) cardioversion. The diagnosis was suspected by transthoracic echocardiography (TTE) and established by cardiac magnetic resonance (CMR). The patient underwent implantable cardioverter–defibrillator (ICD) implantation for secondary prevention of VT, and he was discharged safely on antiarrhythmic drugs with regular follow-up visits.
EMF presenting with VT are quite rare and to the best of our knowledge, our case is the fourth case in the literature to report VT as a clinical presentation of EMF.
KeywordsEndomyocardial fibrosis Ventricular tachycardia Cardiac magnetic resonance Echocardiography
Arrhythmogenic right ventricular dysplasia
Cardiac magnetic resonance
Direct current cardioversion
Left bundle branch
Late gadolinium enhancement
Right bundle branch
- VA, VT
Ventricular arrhythmias and tachycardias
Endomyocardial fibrosis (EMF) is a progressive disease of unknown origin affecting children and young adults in African countries. Heart failure and supraventricular tachycardias are the main symptoms . Ventricular arrhythmia (VT) is rarely encountered in EMF and only a few case reports exist [1, 2]. This report describes a very rare presentation of EMF with recurrent attacks of VT highlighting the previously published cases with such peculiar presentation.
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EMF is a progressive disease that affects children and young adults in African countries with equal sex predilection . Multiple factors have been implemented in the pathogenesis, such as eosinophilia, parasitic infections and environmental, genetic, and immunologic factors. Nonetheless, the exact etiology of EMF remains to be unknown. EMF is characterized by fibrous endocardial involvement of the inflow of the right or left ventricle or both and often involves in atrioventricular valves resulting in regurgitation . Bi-ventricular disease occurs in about 50% cases with pure left ventricular affection in 40% and pure RV involvement in the remaining 10% of cases . EMF is usually associated with heart failure symptoms and supraventricular tachycardias as the main symptoms.
Criteria for diagnosis and assessment of the severity of endomyocardial fibrosis 
Endomyocardial plaques > 2 mm in thickness
Thin (≤ 1mm) endomyocardial patches affecting more than one ventricular wall
Obliteration of right ventricular or left ventricular apex
Thrombi or spontaneous echo contrast without severe ventricular dysfunction
Retraction of the right ventricular apex (right ventricular apical notch)
Atrioventricular-valve dysfunction due to adhesion of the valvular apparatus to the ventricular wall (the score is assigned according to the severity atrioventricular regurgitation)
Thin endomyocardial patches localized to one ventricular wall
Restrictive flow pattern across mitral or tricuspid valves
Pulmonary-valve diastolic opening
Diffuse thickening of the anterior mitral leaflet
Enlarged atrium with normal-size ventricle
M-movement of the interventricular septum and flat posterior wall
Enhanced density of the moderator or other intraventricular bands
Nowadays, multi-modality cardiac imaging including CMR became a must to confirm the diagnosis of rare types of cardiomyopathies including EMF .
Given that the available literature is limited to case series that do not fully define treatment regimens, EMF is treated like other restrictive cardiomyopathies with diuretics and rate control for atrial fibrillation. In cases with advanced heart failure, endomyocardial resection with valve replacement or repair offered better long-term survival. However, high immediate postoperative mortality was reported. Moreover, given the lack of controlled studies, it is still not clear when is the appropriate timing of the surgery .
All authors shared in the collection of the data and writing the case and all approved the submission of the final manuscript.
No funding was received.
Ethics approval and consent to participate
Consent for publication
The corresponding author had a written consent of the patient to use the data for publication.
The authors declare that they have no competing interests.
- 1.Aggarwal A, Sinha B, Rajpal S, Dwivedi S, Sharma V (2009) Right ventricular endomyocardial fibrosis presenting with ventricular tachycardia and apical thrombus-an interesting presentation. Ind Pacing Electrophysiol J 9(6):360Google Scholar
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