Multimodality imaging analysis of the spectrum of endomyocardial fibrosis involvement in a clinical case series.

Background: Endomyocardial fibrosis (EMF) is a rare restrictive heart disease of the tropics. Its aetiology is cryptogenic; however, some hypotheses have been described. The clinical course is characterized by the presence of three phases (acute, subacute, and chronic). Most of the typical features of the disease are identified in the chronic phase. Multimodality imaging allows a presumptive diagnosis and cardiac magnetic resonance imaging, through its tissue characterization, allows confirmation of the diagnosis. Case summary: We present three clinical cases of patients with EMF, at different stages of the disease, which constituted a diagnostic challenge. However, it is due to multimodality imaging that a timely and accurate diagnosis is achieved. Discussion: Endomyocardial fibrosis is a rare heart disease with a restrictive phenotype and a poor prognosis. Endomyocardial fibrosis is characterized by apical obliteration with fibrous tissue, of one or both ventricles, and is usually associated with thrombosis, calcification, and AV valve insufficiency. Transthoracic echocardiogram is the first-line imaging modality to assess for EMF. Cardiac magnetic resonance, through late gadolinium enhancement, allows tissue characterization and identifies the 'double V sign', which is pathognomonic of the disease, allowing confirmation of the diagnosis. Therefore, multimodality imaging is essential for the initial and definitive diagnosis of this disease.