title: Bacteremia in Patients with Heterotaxy: A Review and Implications for Management.
abstract: Heterotaxy (HTX) is a laterality defect resulting in abnormal arrangement of the thoracic and abdominal organs across the right-left axis, and is associated with multiple anatomic and physiologic disruptions. HTX often occurs in association with complex congenital heart disease. Splenic abnormalities are also common and convey an increased risk of bacteremia (bacteremia) with a high associated mortality. We performed a systematic review of the literature studying the risk of infection in HTX patients and strategies that can be utilized to prevent such infections. Studies were identified for inclusion using PubMed, EMBASE, and OVID, as well as hand search of references from previously identified papers. Published studies specifically investigating bacteremia in HTX were identified and included as long as they were in English. Data were extracted by two separate authors independently with review of any findings that differed between the two authors. There were 42 documented cases of bacteremia in 32 patients. Approximately, 79% of these had absence of a spleen. The average age of bacteremia was 17 months. HTX patients are at high risk for bacteremia leading to mortality, regardless of anatomic splenic type. We propose strategies for the evaluation of splenic function in HTX patients, and review management practices to reduce the impact of infection risk in the HTX population.
authors.affiliation: Division of Cardiology, Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, Wis, USA.
authors.forename: Rohit S
authors.initials: RS
authors.lastname: Loomba
authors.affiliation: Department of Genetics, Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, Wis, USA.
authors.forename: Gabrielle C
authors.initials: GC
authors.lastname: Geddes
authors.affiliation: Department of Genetics, Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, Wis, USA.
authors.forename: Donald
authors.initials: D
authors.lastname: Basel
authors.affiliation: Division of Cardiology, Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, Wis, USA.
authors.forename: Dudley W
authors.initials: DW
authors.lastname: Benson
authors.affiliation: Divisions of Neonatology, Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, Wis, USA.
authors.forename: Steven R
authors.initials: SR
authors.lastname: Leuthner
authors.affiliation: Division of Cardiology, Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, Wis, USA.
authors.forename: David A
authors.initials: DA
authors.lastname: Hehir
authors.affiliation: Critical Care, Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, Wis, USA.
authors.forename: Nancy
authors.initials: N
authors.lastname: Ghanayem
authors.affiliation: Division of Cardiology, Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, Wis, USA.
authors.forename: Amanda J
authors.initials: AJ
authors.lastname: Shillingford
mesh_terms: D016470:Bacteremia / Q000175:diagnosis / Q000382:microbiology / Q000401:mortality / Q000628:therapy*; D001424:Bacterial Infections / Q000175:diagnosis / Q000382:microbiology / Q000401:mortality / Q000628:therapy*; D059446:Heterotaxy Syndrome / Q000175:diagnosis / Q000382:microbiology / Q000401:mortality / Q000628:therapy*; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D011379:Prognosis; D012307:Risk Factors; D013154:Spleen / Q000002:abnormalities*
keywords: Antibiotic; Asplenia; Bacteremia; Fever; Heterotaxy; Isomerism; Polysplenia; Prophylaxis