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.