Angina-Like Chest Pain and Syncope as the Clinical Presentation of Left Ventricular Endomyocardial Fibrosis

Bestetti, Reinaldo B.; Corbucci, Hélio A.R.; Fornitano, Luis Domingos; Godoy, Moacir Fernandes de; Cury, Patrí­cia Maluf; Villafanha, Daniel Fernando; Santana, Domingos A.; Soares, Marcelo José Ferreira; Braile, Domingo Marcolino

doi:10.1177/000331970505600316

Cited by 2 publications

(2 citation statements)

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“…The endocardial ventricular thickening detected on echocardiography corresponded to the thick whitish endocardial walls and the valve apparatus surgically observed in variable extensions. The atypical chest pain complaints which were mostly described by the much older children in our study has been mentioned by some authors and linked to the pathological alterations in the left ventricle (19)(20)(21). Although, we were unable to characterize the chest pain complaints made by the children; the clinical impression is that, it might be a result of an altered cardiac microcirculation.…”

Section: Discussionmentioning

confidence: 60%

Endomyocardial fibrosis

Iroegbu

Chen

et al. 2020

Cardiovasc Diagn Ther

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Background: Endomyocardial fibrosis (EMF) is a neglected cardiovascular disease of poverty which carries a poor prognosis with no specific treatment affecting mainly children and young adults. Here, we report our 10-year experience in the therapeutic management and surgical treatment for EMF.Methods: From February 2009 to 2019 March, 55 patients diagnosed with EMF from our cardiology unit underwent surgical repair at our department's pediatric surgical division. There were 35 male, and 20 female patients whose ages varied from 1 year 2 months to 12 years mean age 5.7 (±3.2). We designed the study aimed at assessing the cardio-structural abnormalities and coronary vascular changes faced with EMF patients using echocardiography, and coronary angiography with a detailed and thorough surgical examination of each case.Results: Of the 55 operated patients, 1 had mild lesions, 26 had moderate lesions, and 28 had severe heart disease. All but one patient was in NYHA functional class III or IV at the time of surgery. All but one female patient with mild ventricular lesions and no valvular involvement had severe atrioventricular valve regurgitation with valves considered suitable for both replacements; 45 patients mean age 6.0 (±3.1) and repair nine patients mean age 3.8 (±2.9). The mean endocardial thickness was 3,000 (±1519) µm. Conclusions:The echocardiographic changes corresponded well to the findings on surgery and histopathology. The coronary changes seen included a spectrum of fibrin deposition, medial sclerosis and degeneration, and the formation of plexiform lesions. Surgically evaluating the resected cardiac tissue might help improve disease management.

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Section: Discussionmentioning

confidence: 60%

Endomyocardial fibrosis

Iroegbu

Chen

et al. 2020

Cardiovasc Diagn Ther

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“…Atypical chest pain in EMF has been mentioned by some authors and was linked to pathological alterations in the left ventricle [34][35][36]. Chest pain was present in 50% of the patients studied, sometimes during the follow-up, all of them presenting left ventricular disease.…”

Section: Discussionmentioning

confidence: 96%

Endomyocardial fibrosis: pathological and molecular findings of surgically resected ventricular endomyocardium

et al. 2008

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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 thickening of the endocardium and adjacent myocardium. Surgical treatment is recommended for patients in functional classes III or IV (New York Heart Association). The gross and histological features of the heart have been comprehensively studied in autopsies, but studies in surgical samples are still lacking. Histological and immunohistochemical features of EMF in surgical samples collected from 32 patients were described and correlated with clinical data. Polymerase chain reaction (PCR) and reverse transcription-PCR, performed on formalin fixed endomyocardial samples, were used retrospectively to detect genomes of certain cardiotropic viruses and Toxoplasma gondii. Ventricular endocardium was thickened by superficial acellular hyaline collagen fibers type I and III, with predominance of the former type. Besides fibrosis, a chronic inflammatory process and an anomalous lymphatic rich vascular pattern were observed in the deep endocardium, connected to the terminal coronary circulation of the myocardium, which might be an important pathological finding concerning EMF pathogenesis. Molecular analysis of the endomyocardium revealed high incidence of cardiotropic infective agents (6/12, 50%); however, their role in the disease pathogenesis is still controversial.

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Angina-Like Chest Pain and Syncope as the Clinical Presentation of Left Ventricular Endomyocardial Fibrosis

Cited by 2 publications

References 10 publications

Endomyocardial fibrosis

Endomyocardial fibrosis

Endomyocardial fibrosis: pathological and molecular findings of surgically resected ventricular endomyocardium

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