CASE
A 26-year-old asymptomatic female was referred to our hospital for a further evaluation of an abnormal shadow on chest X-ray. The first TTE showed the dilated right atrium and ventricle without significant valvular disease, while intraventricular septum was displaced to the left ventricle predominantly in diastole (Figure 1 A - B, Movie Clip S1). The estimated pulmonary to systemic flow ratio (Qp/Qs) was 2.4, suggesting a significant increase in pulmonary blood flow. These results strongly suggested right heart volume overload disease, especially of the pre-tricuspid left to right shunt disease. However, the first TTE could not reveal none of such diseases.
Contrast-enhanced computed tomography (CT) showed a superior sinus-venosus ASD complicated by PAPVC where the upper right and lower pulmonary veins were connected to the superior vena cava (SVC), and to the right atrium (Figure 2 A - D). A right heart catheterization with selective angiography of the right pulmonary artery also showed anomalous connection of the right pulmonary veins to the right heart. Oxygen saturation stepped up in the right upper atrium and Qp/Qs was 2.8. We performed a second TTE with a more complete understanding of the anatomical relationship identified by the CT, transesophageal echocardiography (TEE), and angiography. An atrial septum defect, with a diameter of approximately 7 mm, was observed around the ceiling of the atrial septum. When the probe was gently tilted to the left heart system from the right ventricular inflow view projected at the apical long-axis view (Figure 3 A - C, Movie Clip S2 A - B), we showed the upper and lower right pulmonary veins connected separately to the SVC and to the right atrium (RA), respectively. She successfully underwent surgical intracardiac repair. Intraoperative findings revealed that the upper right pulmonary vein returned to the vicinity of the junction between the SVC and the right atrium. The atrial septal defect was enlarged, while rerouting of the anomalous pulmonary veins to the left atrium was performed with an autologous pericardial baffling.