The prenatal finding of abdominal heterotaxia in conjunction with complex congenital heart defects should indicate the possible diagnosis of cardiosplenic syndrome—specifically, asplenia or polysplenia. Unfortunately, the prognosis for patients with either of these syndromes is usually poor. In this case, antenatal findings were indicative of asplenia, but neonatal testing revealed a normal-functioning spleen. This finding, along with detailed documentation of the congenital heart defects by fetal echo examination, dramatically improved the patient's prognosis and ultimate outcome.
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