Abstract 15116: The Incidence of Severe Bacterial Infections in Asplenia and Polysplenia Syndrome: A Multi-Center Study
Backgrounds: Surgical splenecomy is known to increase the risk of bacterial infections (BI). However, the rate of BI in heterotaxy syndrome (congenital asplenia and polysplenia) has not been studied. In Japan, pneumococcal vaccination has started only recently and daily antimicrobial prophylaxis has not been performed routinely both in asplenia (AS) and polysplenia syndrome (PS). The purpose of this study was to evaluate the risk of BI in pts with AS and PS and to evaluate if the risk of BI is lowered by vaccination and/or antimicrobial prophylaxis.
Methods: Clinical information regarding BI was evaluated retrospectively at 16 institutions from the charts of 641 pts with AS, 370 pts with PS, and 527 control pts without heterotaxy but with single ventricle or double outlet right ventricle. All AS and PS pts had congenital heart diseases. A total observation of 4643 patient-years in AS, 4026 patient-years in PS, and 5598 patient-years in control was derived. The diagnosis of AS was based on the presence of right isomerism, absence of spleen by imaging, and/or presence of Howell-Jolly bodies in red blood cells. The diagnosis of PS was based on the presence of left isomerism and/or multiple spleens by imaging. BI was defined as sepsis, meningitis, and/or sudden death due to overwhelming infections.
Results: In AS, BI were observed in 20% of pts (274/10,000 patient-years) and pneumococcus was responsible in 24 % of BI. In AS, death due to overwhelming BI was observed in 7% of pts (95/10,000 patient-years). In PS, the incidence of BI (82/10,000 patient-years) was significantly lower than in AS, was similar to that in pts with aortic stenosis (85/ 10,000 patient-years) (Circ 87; supple I-121, 1993), but was significantly higher than that in control group (48/10,000 patient-years). BI by pneumococcus and death due to overwhelming BI were not observed in PS and in control group. In AS, the rate of BI in pts who had pneumococcal vaccination (8%), and in pts who had daily antimicrobial prophylaxis (2%, 4/10,000 patient-years) was significantly lower than in pts without these prophylaxis (20%).
Conclusions: The risk of BI in AS is high and vaccination and daily antimicrobial prophylaxis are recommended. The risk of BI in PS may not be as high as to warrant daily antimicrobial prophylaxis.
- © 2011 by American Heart Association, Inc.