Caught-off guard: Unguarded mitral valve orifice in usual atrial arrangement with discordant atrioventricular connections and pulmonary atresia

Caught-off guard: Unguarded mitral valve orifice in usual atrial arrangement with discordant atrioventricular connections and pulmonary atresia:

Nayan Banerji, Mani Ram Krishna, Raman Krishna Kumar, Robert H Anderson



Annals of Pediatric Cardiology 2020 13(1):84-86



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.