Fatal pneumococcal septicaemia associated with asplenia and isomerism of the right atrial appendages.

A 22 month old male infant with isomerism of the right atrial appendages (associated with a complete atrioventricular septal defect, double outlet right ventricle, and pulmonary valve stenosis) died from pneumococcal septicaemia after a 36 hour illness. He had not been given penicillin prophylaxis. Asplenia was confirmed at necropsy. In a survey of 50 paediatric cardiologists in the United Kingdom, 13 (33%) of the 40 (80%) who replied did not advise any prophylactic measures against pneumococcal infection in patients with isomerism of the right atrial appendages.