The authors describe the case of a 54-year-old woman with the association of two rare congenital cardiac anomalies: cor triatriatum and persistent left superior vena cava with the special characteristic of direct drainage to the right atrium. Real-time three-dimensional transesophageal echocardiography offered a comprehensive anatomic and functional evaluation of these infrequent entities.
Cor triatriatum (CT) is a rare congenital heart disease. This anomaly is frequently associated with other congenital cardiac disorders, such as the presence of a persistent left superior vena cava (PLSVC).
We describe the case of CT associated with a PLSVC diagnosed by transthoracic echocardiography and confirmed by real-time three-dimensional transesophageal echocardiography, which facilitated complete anatomic diagnosis.
Case Presentation
A 54-year-old woman underwent echocardiography as part of the diagnostic workup after a suspected transient ischemic attack. Transthoracic and transesophageal echocardiography showed a membrane dividing the left atrium into two chambers. Antegrade flow through the membrane was observed by color Doppler echocardiography ( Figure 1 ).
Additionally, a channel was observed in the lateral portion of the left atrium. After injection of agitated saline into the left antecubital vein, contrast was seen first in the channel and later in the right atrium, demonstrating a PLSVC draining to this venous collector ( Figure 2 ).
Real-time three-dimensional transesophageal echocardiography was performed, confirming the existence of double left atrial chambers with a posterior-superior chamber, which received the pulmonary veins, and another anteroinferior chamber, relating to the left atrial appendage and communicating with the mitral valve orifice. The intermediate membrane presented a single fenestration in its posteromedial portion with an area of 1.4 cm 2 by planimetry, showing no obstructing gradient ( Figure 3 , Video 1 ). The venous collector behind the left atrium leading from the area between the upper portion of the left atrial appendage and the left superior pulmonary vein drained directly to the right atrium, but with a different trajectory than the coronary sinus, so that it was not dilated ( Figures 4 and 5 ). Again, after injection into the left antecubital vein, saline contrast was seen first in the venous collector and then in the right atrium ( Video 2 ). Thus, an accurate diagnosis of CT associated with a PLSVC with direct drainage to the right atrium could be established.
