Unguarded mitral valve orifice is a rare disease with only 7 described cases in the literature. We describe the first known case of unguarded mitral valve orifice with normal segmental cardiac anatomy, severe left ventricular dilatation and dysfunction, aortic atresia, and atrial flutter. (Level of Difficulty: Advanced.).
Keywords: aortic atresia; atrial flutter; congenital heart defect; fetal; heart malformation; unguarded mitral valve orifice.