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.
D001784:Blood Gas Analysis; D001794:Blood Pressure; D005260:Female; D006330:Heart Defects, Congenital / Q000097:blood / Q000150:complications* / Q000503:physiopathology; D006801:Humans; D007231:Infant, Newborn; D013154:Spleen / Q000002:abnormalities*; D013577:Syndrome