Situs ambiguous anomaly is a rarely encountered condition in clinical practice that is characterized by the presence of multiple congenital anomalies relevant to intraabdominal organs and the cardiovascular system. While this syndrome is mostly diagnosed as a serious cyanotic cardiac disease in the first year of life, only 5% may survive beyond five years of life, and it can be a diagnostic challenge. In this report, we present an adult case of situs ambiguous anomaly which was diagnosed incidentally. The patient had centrally located liver, multiple splenules, interrupted inferior vena cava with azygos continuation, and bilateral bilobed lungs. Furthermore, she had a history of an atrial septal defect operation 20 years previously. These congenital anomalies were found to be compatible with situs ambiguous anomaly (its polysplenia variant). The interesting points of this patient are that she reached an advanced age without complaint in spite of congenital heart disease, and that the diagnosis was made incidentally during routine examination.
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