Cor triatriatum dexter is a rare cardiac abnormality in which the right atrium is subdivided into two distinct chambers. This anomaly is generally attributed to the persistence of the right sinus venosus valve and it is frequently associated with severe malformations of other right heart structures.
During embryogenesis, the right horn of the sinus venosus gradually incorporates into the right atrium to form the smooth posterior portion of the right atrium. The original embryologic right atrium forms the trabeculated anterior portion. The connection between the right horn of the sinus venosus and the embryologic right atrium is the sinoatrial orifice, which is flanked on either side by two valvular folds, the right and left venous valves. At some point during this incorporation, the right valve of the right horn of the sinus venosus divides the right atrium in two. This right valve forms a sheet that serves to direct the oxygenated venous return from the inferior vena cava across the foramen ovale to the left side of the heart during fetal life. The valve usually regresses by the 12th week of gestation leaving behind the crista terminalis superiorly and the eustachian valve of the inferior vena cava and the thebesian valve of the coronary sinus inferiorly. There can be remnants or patritions. There are those with filigreed weblike networks as remnants of the right valve (also known as Chiari network) and those with a persistent partition between the venous (smooth) and trabeculated portions of the right atrium - cor triatriatum dexter.
Once the diagnosis is established, the condition is amenable to a relatively simple surgical correction.
Case report with MRI images.
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