Deviation of atrial septum primum in association with normal left atrioventricular valve size

Deviation of atrial septum primum (DASP) is an unusual anatomic finding in patients with normal left atrioventricular valve size. To better describe this anomaly, we reviewed our echocardiographic database from January 1987 to May 1999. Twenty-four patients had DASP and a normal-sized left atrioventricular valve. Six had inferior DASP with common atrioventricular canal. Of these, 3 had evidence of left atrial outlet obstruction. Five survived surgical intervention with follow-up of 24 +/- 14 months. Seventeen patients had superoposterior deviation with the right pulmonary veins draining anomalously to the right atrium. Only 8 of 17 underwent surgical resection; none of the 17 have evidence for late development of obstruction. One patient had both inferior deviation and superoposterior DASP and underwent early successful repair. DASP occurs in patients with normal left atrioventricular valve size. Inferior DASP can result in left atrial outlet obstruction and requires surgical intervention. Superoposterior DASP carries less physiologic consequence. Echocardiography can distinguish this anomaly from other forms of left atrial obstruction.