A rare case of neonatal cyanosis due ‘cor triatriatum dexter’ and a review of the literature

Abstract

Cor triatriatum dexter is a rare cardiac congenital malformation in which a membrane divides the right atrium into two chambers. We describe one case of isolated cor triatriatum dexter, symptomatic for severe central cyanosis, in which the membrane was identified before surgery by means of transthoracic echocardiography alone, and successfully removed surgically at 1 month of life. We discuss this case and review the literature.

Cor triatriatum dexter with right ventricular hypoplasia: Role of multimodality imaging in decision making

“…With mild degrees of obstruction, clinical symptoms may be absent and this may be discovered only on postmortem examination . Severe obstruction on the other hand can result in severe central cyanosis with the abnormal membrane obstructing the IVC as it enters into the right atrium, at the TV orifice or the right ventricular outflow tract leading to a predominant right‐to‐left shunt as seen in our case . It can cause elevated central venous pressure manifesting as jugular venous distension and hepatic congestion due to functional tricuspid stenosis.…”

“…In fetal life, CTD can cause growth restriction of right heart structures and is frequently encountered with other defects such as PS and hypoplastic RV as seen in our patient . Some theories for RV hypoplasia include decreased blood flow to the RV due to the supratricuspid membrane . Echocardiography and cMRI are the preferred imaging modalities to delineate the anatomy of this complex membrane.…”

“…Other complications include embolic phenomena, catheter entanglement, and supraventricular arrhythmias such as atrial flutter. 1,3 In fetal life, CTD can cause growth restriction of right heart structures 1,4,5 and is frequently encountered with other defects such as PS and hypoplastic RV as seen in our patient. 4 Despite the presence of CTD causing moderate RV hypoplasia in our patient, after closure of the ASD and resection of the membrane, there were no signs of right heart failure and the RV was able to handle the entire cardiac output, enabling a successful biventricular repair.…”

“…1,3 In fetal life, CTD can cause growth restriction of right heart structures 1,4,5 and is frequently encountered with other defects such as PS and hypoplastic RV as seen in our patient. 4 Despite the presence of CTD causing moderate RV hypoplasia in our patient, after closure of the ASD and resection of the membrane, there were no signs of right heart failure and the RV was able to handle the entire cardiac output, enabling a successful biventricular repair. This physiology is not akin to hypoplastic right heart syndrome with pulmonary atresia and intact ventricular septum, wherein the right ventricle is heavily trabeculated and muscle bound and the size of the tricuspid valve and RV dependent coronary circulation play a very important role in determining the optimal surgical approach.…”

Cor Triatriatum Dexter as an Incidental Finding: Role of Two-Dimensional Transthoracic Echocardiography

“…The upstream chamber receives venous blood from the caval system and the coronary sinus, while the downstream chamber incorporates the tricuspid valve and right atrial appendage [4]. This membrane is a reminiscent structure formed due to the persistence of the right valve of the sinus venosus, an early embryonic structure that usually recedes between the ninth and the fifteenth week of gestation [1,5].…”