Polysplenia syndrome. A study of five new cases.

Five children with polysplenia syndrome are described. Cardiac catheterization or postmortem examination revealed the following cardiac anomalies: interruption of the inferior vena cava with azygos (or hemiazygos) continuation to the left superior vena cava and a single atrium or a large atrial septal defect in all five children; a ventricular septal defect in three; and a primitive ventricle in one case. Other anomalies found were: central liver in all five; a right-sided stomach in three; and multiple small spleens and bilateral left, bilobed lungs--found on autopsy--in two of the children. In four of the five patients, whose ECG was available, a negative P wave was present in leads II, III and AVF. This leftward and superiorly directed P wave axis should suggest a diagnosis of polysplenia syndrome in an infant with congenital heart disease. The cardiac anomalies are surgically correctable; therefore, early recognition of this syndrome is of practical importance.