Three cases of asplenia syndrome associated with congential heart disease

The asplenia and polysplenia syndromes are characterized by visceral situs ambiguus because of more or less pronounced isomerism of normally asymmetric organs such as lungs, atria and liver. The asplenia syndrome is the more frequently recognized of the two and has a high tendency toward 'bilateral right-sidedness'. Complex and multiple cardiovascular anomalies are the rule in asplenia syndrome and almost always they are cyanotic. This complexity of cardiac malformation determines the clinical course and prognosis, and frequently precludes surgical correction. Therefore it is very important to recognize the patients with asplenia syndrome and distinguish them from other forms of cyanotic congenital heart disease. Authors present 3 cases of asplenia syndrome with congenital heart disease in which cardiac catheterization and angiocardiography were done at the Department of Radiology, Seoul National University Hospital