A Rare Association of Sinus Venosus-Type Atrial Septal Defect and Persistent Left Superior Vena Cava Detected by Transthoracic Echocardiography and Cardiac Magnetic Resonance Imaging

BACKGROUND: Association of persistent left superior vena cava (PLSVC) and sinus venosus-type atrial septal defect (SVASD) is rare. We describe a patient with dilated coronary sinus (CS) found to have PLSVC and SVASD.
CASE REPORT: The patient is a 60-year-old man with history of stroke who underwent a transthoracic echocardiogram (TTE) for evaluation of shortness of breath. TTE demonstrated a markedly dilated CS. Agitated saline was injected into the left antecubital vein to further assess CS. The parasternal long axis view demonstrated immediate filling of the CS and confirmed the presence of a PLSVC. Apical 4-chamber view with injection of agitated saline into the right antecubital vein demonstrated immediate contrast opacification of both atria, consistent with a right to left cardiac shunt. Cardiac magnetic resonance (CMR) was performed, which confirmed the TTE findings of PLSVC and defined the cardiac shunt as SVASD.
CONCLUSIONS: PLSVC should be suspected in a patient with an abnormally dilated CS. In this case we identified a rare association of PLSVC with a SVASD. TTE with agitated saline contrast injection and CMR are useful diagnostic tools for PLSVC and associated cardiac congenital anomalies, respectively.

Keywords: Heart Septal Defects, Atrial - diagnosis, Echocardiography, Magnetic Resonance Imaging, Vena Cava, Superior - abnormalities