A Misdiagnosed Case of Double Outlet Right Atrium Associated With Hypoplastic Right Ventricle. Issue 3 (May 2020)

• Record Type: Journal Article
• Authors: Raso, Irene; Quarti, Andrea; Chessa, Massimo; Basile, Domenica Paola; Saracino, Antonio; D'Aiello, Angelo Fabio; Raineri, Claudia; Carminati, Mario; Giamberti, Alessandro
• Is Part Of: World journal for pediatric & congenital heart surgery. Volume 11:Issue 3(2020)
• Page Start: 358
• Page End: 360
• Publication Date: 2020-05
• Subjects: Pediatric cardiology -- Periodicals; Congenital heart disease in children -- Periodicals; Heart -- Abnormalities -- Surgery -- Periodicals; Heart -- Surgery -- Periodicals; Heart Defects, Congenital -- surgery -- Periodicals; Cardiac Surgical Procedures -- Periodicals; Child -- Periodicals; Adult -- Periodicals; 618.9212
• DOI: 10.1177/2150135119894292
• Languages: English
• ISSNs: 2150-1351

Abstract

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.