Left atrial isomerism combined with right-sided heart, common atrium, incomplete atrioventricular septal defect and partial anomalous systemic and pulmonary venous drainage. Report of a case with successful surgical correction.

Precise preoperative diagnostic clarification was accomplished in an infant with left atrial isomerism. The inner/outer arrangement of the pulmonary/systemic veins in relation to the posterior wall of the common atrium necessitated a specific baffle in order to prevent pulmonary or systemic venous obstruction. The development of a sick sinus syndrome six months after surgery is in keeping with the natural history of the disease.