Incomplete involution of valve of systemic venous sinus can present across a spectrum of anatomical lesions ranging from eustachian valve to division of right atrium (cor triatriatum dexter) with overlapping features. We present the case of a neonate presenting with cyanosis, having persistent valve of systemic venous sinus with anatomical details of the redundant tissue in right atrium suggesting an intermediate form between Chiari network and division of right atrium.

We describe a patient with pulmonary atresia and intact ventricular septum in whom the right atrium was divided by a vascular aneurysm located in the right atrioventricular groove. We postulate that the structure represents an aneursymally dilated right coronary artery taking anomalous origin from the pulmonary trunk, with fistulous communication to the right atrium. We discuss the findings relative to concepts of development of the coronary arteries in normal hearts and in pulmonary atresia with an intact ventricular septum.

We reviewed the echocardiographic features of 16 children with prominent Eustachian valves, six of whom had associated pathology of the right heart, while eight had other anomalies, primarily atrial and ventricular septal defects. The hearts of two patients were structurally normal. Prolapse of the membrane through the orifice of the tricuspid valve or through the atrial septum was present in seven patients, two of whom were symptomatic. One presented with severe cyanosis which resolved after surgical removal on the basis of the echocardiographic findings. The other presented with atrial arrhythmias which did not improve with removal. The size or behavior of the membrane, including prolapse, did not correlate with either age or presence of pathology of the right heart. The echocardiographic appearance of prominent Eustachian valves is variable. Rarely, when prominent, the valves can cause symptoms associated with obstruction to the right heart, particularly cyanosis or an arrhythmia.

We describe the symptoms, the techniques for imaging and the surgical therapy in a patient with divided right atrium (‘cor triatriatum dexter’) combined with atresia of the superior caval vein. In addition to peripheral venous congestion and cyanosis, macrocephaly was the most prominent sign, the high pressure in the superior caval vein disturbing the circulation of cerebrospinal fluid. Transesophageal echocardiography was the optimal technique for imaging the dividing shelf in the right atrium. Cardiac catheterization after surgical excision of the shelf and reconstruction of the junction between the superior caval vein and the right atrium excluded any residual gradient or shunt.