Raso Irene, Quarti Andrea, Chessa Massimo, Basile Domenica Paola, Saracino Antonio, D'Aiello Angelo Fabio, Raineri Claudia, Carminati Mario, Giamberti Alessandro
Department of Cardiology, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy.
Department of Congenital Cardiac Surgery, Azienda Ospedaliero-Universitaria di Bologna, Policlinico Sant'Orsola-Malpighi, Bologna, Italy.
World J Pediatr Congenit Heart Surg. 2020 May;11(3):358-360. doi: 10.1177/2150135119894292.
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.
右心房双出口（DORA）是一种罕见的先天性心脏病，其特征为右心房与两个心室均相连。室间隔向左运动减弱导致心脏十字交叉处心房与室间隔不对齐，这是该心脏缺陷的特征性表现。我们报告一例成年患者，诊断为右心房双出口、右心室受限及三尖瓣异常，出现明显运动性血氧饱和度降低。随后，该患者接受了一期半心室姑息手术。
