Summary
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.
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Berman, W., Yabek, S.M., Burstein, J. et al. Asplenia syndrome with atypical cardiac anomalies. Pediatr Cardiol 3, 35–38 (1982). https://doi.org/10.1007/BF02082329
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DOI: https://doi.org/10.1007/BF02082329