Cor triatriatum dexter variant identified by critical congenital heart disease screening

Abstract

Critical congenital heart disease (CCHD) screening utilizes pulse oximetry to detect heart disease in the neonatal period. While the primary targets for screening are common cyanotic lesions, screening may result in a diagnosis of rare cyanotic lesions such as cor triatriatum dexter (CTD). CTD is a rare congenital cardiac malformation caused by persistence of the right valve of the sinus venosus, which divides the right atrium into two chambers. Here, we describe an asymptomatic female newborn with a positive screening for CCHD diagnosed with a variant of CTD. To the best of our knowledge, this is the first report of CTD diagnosed after a positive CCHD screen. With the advent of CCHD screening, identification of rare congenital heart diseases in asymptomatic infants may increase. CCHD screening allowed diagnosis and management without surgical repair of CTD in a clinically asymptomatic infant.