Double-Outlet Right Atrium: Anatomic and Clinical Considerations

Background Double-outlet right atrium (DORA) is a condition in which the right atrium empties into both ventricles. Described are the anatomic and clinical findings of this rare anomaly and our experience with its surgical treatment. Methods A retrospective review was conducted of all medical records and two-dimensional echocardiographic assessments, and the operative reports of patients with DORA were reexamined to identify the specific anatomic features and the relative adaptation of surgical technique. Results From January 1983 to November 2004, 12 patients with DORA underwent surgical treatment. The diagnosis was made in all cases by two-dimensional echocardiography. All patients had mild-to-moderate signs of arterial oxygen desaturation. All patients also had either a partial (n = 11) or a complete (n = 1) atrioventricular canal with or without other associated cardiac anomalies, including persistent left superior vena cava. Eleven patients survived the surgical repair, the only death occurred in a patient with Ellis Von-Creveld syndrome, with multiple skeletal malformations, severe chest hypoplasia, and respiratory distress. Conclusions DORA is a rare and peculiar cardiac anomaly with well-defined characteristics. Its recognition, best achieved by two-dimensional echocardiography, is crucial for a good surgical correction.