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Double-outlet right atrium in an asymptomatic 8-year-old girl.

An asymptomatic 8-year-old girl was admitted to the outpatient clinic for further evaluation of a previously detected heart murmur. A physical examination was unremarkable with the exception of a 2/6 systolic murmur heard at the left second intercostal space. The oxygen saturation level recorded was 95% and the heart rate was 95 beats per minute. An electrocardiogram revealed a normal sinus rhythm with a superior QRS axis and a left anterior hemiblock pattern. A transthoracic echocardiographic examination demonstrated a common atrioventricular (AV) junction with a leftward rotation of the atrial septum resulting the right atrium draining into both ventricles (double-outlet right atrium [DORA]). Pulmonary venous drainage from the left atrium was transiting to the systemic ventricle through the right atrium via a 10-mm primum-type interatrial defect (Fig. A-D and videos). The cardiac catheterization results were saturation from the superior vena cava: 65%, pulmonary artery (PA): 85%, aorta: 96%; PA pressure: 45/22 mmHg (mean: 33 mmHg); and pulmonary vascular resistance index: 2.4 WU.m2. A diagnosis of DORA was confirmed during surgical correction. DORA is a rare congenital anomaly defined by a defect in atrioventricular septal development in which the right atrium opens into both the right and left ventricles. Three anatomical requisites should be present to classify a case as DORA associated with atrioventricular canal defect. First, there is a peculiar leftward deviation of the “true” atrial septum, a distinctive feature of a cor triatriatum. Second, the presence of a cephalic and posterior left atrial chamber receiving all 4 pulmonary veins and with a normally located left atrial appendage can be observed. Finally, specific anatomical features of the AV valves, consisting of either 2 separate patent valves or a common AV valve, are present. 86