Comprehensive Assessment of Endomyocardial Fibrosis with Cardiac MRI: Morphology, Function, and Tissue...

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 sub-Saharan Africa, South Asia, and South America. EMF is characterized by apical filling with fibrotic tissue of one or both ventricles, often associated with thrombus, calcification, and atrioventricular valve regurgitation, leading to typical symptoms of restrictive heart failure. Transthoracic echocardiography (TTE) is the first-line modality for assessment of EMF, basically owing to its widespread availability. However, in recent years cardiac MRI has emerged as a powerful tool for assessment of cardiac morphology and function, with higher accuracy than TTE, along with the unique advantage of being able to provide comprehensive noninvasive tissue characterization. Delayed enhancement (DE) imaging is the cornerstone of cardiac MRI tissue characterization and allows accurate identification of myocardial fibrosis, conveying valuable additional diagnostic and prognostic information. The typical DE pattern in EMF, described as the "double V" sign, consists of a three-layered pattern of normal myocardium, thickened enhanced endomyocardium, and overlying thrombus at the apex of the affected ventricle; it has excellent correlation with histopathologic findings and plays an important role in differentiating EMF from other cardiomyopathies. Conversely, fibrous tissue deposition quantified using DE imaging, when indexed to body surface area, has been shown to be a strong independent predictor of mortality. The aim of this review is to summarize state-of-the-art applications of cardiac MRI for diagnostic and prognostic assessment of patients with suspected or confirmed EMF. Online supplemental material is available for this article. ^©RSNA, 2020.