Asplenia syndrome with atypical cardiac anomalies

Two infants with asplenia and complex cyanotic congenital heart disease showed unusual findings at cardiac catheterization. Each had a patent pulmonary outflow tract and a severe obstructive lesion of the aortic arch. These cases emphasize the heterogeneity of the asplenia-polysplenia syndromes. Pulmonary atresia or critical pulmonic stenosis is not an invariable accompaniment of the asplenia syndrome. Obstructive lesions of the aortic outflow tract, which occur in about 1/3 of patients with the polysplenia syndrome, also can occur with asplenia.