[Atresia of the left atrioventricular orifice with "normal" aorta (author's transl)]

Though atresia of the left atrioventricular (av) orifice can show multiple types of ventricular morphology and different ventriculo-arterial (va) connections, clinical prognosis and surgical reliability depend upon the size of the aorta. Two cases with "normal" aorta are reported. The first was a female baby, died at four months, who had atresia of the left av orifice with right main ventricular chamber, va discordance and moderate pulmonary stenosis. The fatal outcome was due to progressive restriction of the foramen ovale. The second patient was studied at six months, is still alive, and showed atresia of the left av orifice with left main ventricular chamber, va discordance and pulmonary blood overload. According to this type of anatomy, modified Fontan procedure is postulated as possible and reliable surgical correction.