Can echocardiography predict the presence or absence of endocardial fibroelastosis in infants < 1 year of age with left ventricular outflow obstruction?

Endocardial fibroelastosis (EFE) is a myocardial histologic diagnosis that is frequently associated with congenital left-sided obstructive anomalies. When present, ventricular dysfunction is manifested, which may render the left ventricle unusable as the systemic ventricle. Reliably detecting EFE noninvasively is important because it is a factor that will contribute to determining the appropriate surgical strategy to pursue. If left ventricular EFE is absent, then structural relief of obstruction may be considered. However, if EFE is present, then a single ventricle approach via the Norwood operation should be undertaken. The use of echocardiography in determining the presence or absence of EFE has not been rigorously evaluated. A high degree of ultrasonic reflectivity of the endocardial surface, as well as a spherical shape to the left ventricle, have been postulated as findings consistent with EFE. 1. Sharland G Chita S Fagg N Anderson R Tynan M Cook A Allan L Left ventricular dysfunction in the fetus relation to aortic valve anomalies and endocardial fibroelastosis. Br Heart J. 1991; 66: 419-424 Crossref PubMed Scopus (111) Google Scholar , 2. Revel A Ariel I Rein A Anteby E Lavy Y Yagel S Fetal endocardial fibroelastosis. J Clin Ultrasound. 1994; 22: 355-356 Crossref PubMed Scopus (5) Google Scholar To assess the role of echocardiography in this capacity, we investigated the reliability of (1) endocardial “echo-brightness,” and (2) left ventricular geometric shape in predicting the presence of biopsy-proven EFE.