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.
D000015:Abnormalities, Multiple; D002648:Child; D002675:Child, Preschool; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008099:Liver / Q000002:abnormalities; D008168:Lung / Q000002:abnormalities; D008297:Male; D013154:Spleen / Q000002:abnormalities*; D013270:Stomach / Q000002:abnormalities; D013577:Syndrome