Divided left atrium with totally anomalous drainage of normally connected pulmonary veins

Cardiol Young. 2022 Apr 29:1-3. doi: 10.1017/S1047951121005163. Online ahead of print.


In the December 2021 issue of Cardiology in the Young, Hubrechts and colleagues, from Brussels and Leuven in Belgium, describe their experience in which the pulmonary veins were normally connected to the morphologically left atrium. By virtue of the presence of a shelf dividing the morphologically left atrium, however, the venous return was to the morphologically right atrium, with no evidence of formation of the superior interatrial fold, meaning that there was no obstruction of flow into the systemic venous circulation. The question posed by the Belgian authors is whether the shelf dividing the morphologically left atrium is a deviated primary atrial septum, as the arrangement has previously been interpreted. As they discuss, it is currently impossible to arbitrate this conundrum. In our commentary, we discuss the background to the dilemma. We point out that, as yet, it is not possible to code accurately this congenital cardiac malformation within The International Paediatric and Congenital Cardiac Code (IPCCC), nor within the newly produced 11th Revision of the International Classification of Diseases (ICD-11).

Keywords: Pulmonary venous drainage; cardiac development; cor triatriatum sinister; nomenclature.