Discussion:
UCSS is a rare CHD representing less than 1% of all ASD types (1). Its
clinical manifestation is atypical, and may be associated with varied
CHDs (6). Preoperative diagnosis by TTE is very difficult when
associated with PLSVC (2, 6). In our patient, the diagnosis of
associated PLSVC was missed preoperatively, and was detected
intraoperatively, and this had led to change in the surgical plan. Our
surgical approach was to create an intra-atrial tunnel to divert the
flow of PLSVC into RA with great attention to the pulmonary veins and
the mitral valve, and repair the ASD with fresh autologous pericardial
patch . Other operative options include: ligating PLSVC and repairing
the ASD; constructing a baffle to guide PLSVC towards RA (6). We confirm
the importance of precise preoperative diagnosis to avoid any
complications from such an incidental intraoperative finding.