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.
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.