Unguarding of an atrioventricular valvar orifice is a rare form of congenital heart disease that requires staged functionally univentricular palliation. Unguarding of the mitral valvar orifice has previously been reported in the setting of mirror-imaged atrial arrangement. We report a neonate with unguarding of the mitral valvar orifice in the setting of usual atrial arrangement, but with discordant atrioventricular connections and pulmonary atresia.
Keywords: Atrioventricular discordance; mitral incompetence; pulmonary atresia.
Copyright: © 2019 Annals of Pediatric Cardiology.