A Misdiagnosed Case of Double Outlet Right Atrium Associated With Hypoplastic Right Ventricle


Raso I, Quarti A, Chessa M, Basile DP, Saracino A, D’Aiello AF, Raineri C, Carminati M, Giamberti A.World J Pediatr Congenit Heart Surg. 2020 May;11(3):358-360. doi: 10.1177/2150135119894292.PMID: 32294022



Double outlet right atrium (DORA) is a rare congenital heart disease in which the right atrium opens into both ventricles. The reduced leftward motion of the interventricular septum causes a malalignment between the atrial and the ventricular septum at the cardiac crux, which is the pathognomonic feature of this heart defect. We describe a case of significant exertional desaturation in an adult patient who was diagnosed with DORA, restrictive right ventricle, and anomalous tricuspid valve. Subsequently, the patient underwent one-and-a-half ventricular palliation.