POLYSPLENIA SYNDROME - STUDY OF 5 NEW CASES

  • 1 January 1978
    • journal article
    • research article
    • Vol. 14  (9) , 948-953
Abstract
Children [5] 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 5 children, a ventricular septal defect in 3 and a primitive ventricle in 1 case. Other anomalies found were central liver in all 5; a right-sided stomach in 3 and multiple small spleens and bilateral left, bilobed lungs (found an autopsy) in 2 of the children. In 4 of the 5 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. Early recognition of this syndrome is of practical importance.