title: Extracorporeal membrane oxygenation in children with heart disease and genetic syndromes.
abstract: Our objective was to evaluate morbidity and mortality associated with extracorporeal membrane oxygenation (ECMO) in children with genetic syndromes and heart disease. We conducted a retrospective review of all children with heart disease and genetic syndromes receiving ECMO during the period January 2000 and March 2012 at Arkansas Children's Hospital, Little Rock. The medical charts were reviewed to obtain the following variables: demographic information, medical and surgical history, laboratory and microbiological, information on organ dysfunction, and outcome characteristics. The outcome variables evaluated in this report included: hospital length of stay (LOS), survival to hospital discharge, and current survival. Outcome data were compared among critically ill children with and without syndromes. During the study period, there were 377 ECMO runs in 336 children with heart disease. Of these, 43 ECMO runs occurred in children with genetic syndromes whereas 334 ECMO runs occurred in children with no genetic abnormality. Children in the group with underlying genetic syndrome were older at the time of ECMO cannulation than the group with no syndrome. During the ECMO run, hospital LOS and mortality were similar in children with and without underlying genetic abnormality. Among genetically abnormal patients, renal insufficiency and need for dialysis were associated with mortality. In this group, 24 patients (56%) were discharged alive. However, only 10 patients are living to date in this cohort. ECMO can be used in children with heart disease and genetic syndromes with good results. The survival rate is high and the complication rate is low.
authors.affiliation: Divisions of Pediatric Cardiology, University of Arkansas Medical Center, Little Rock, Arkansas, USA.
authors.forename: Santosh C
authors.initials: SC
authors.lastname: Uppu
authors.forename: Sunali
authors.initials: S
authors.lastname: Goyal
authors.forename: Jeffrey M
authors.initials: JM
authors.lastname: Gossett
authors.forename: Karen
authors.initials: K
authors.lastname: Yan
authors.forename: Duy T
authors.initials: DT
authors.lastname: Dao
authors.forename: Eudice E
authors.initials: EE
authors.lastname: Fontenot
authors.forename: Michiaki
authors.initials: M
authors.lastname: Imamura
authors.forename: Punkaj
authors.initials: P
authors.lastname: Gupta
mesh_terms: D001131:Arkansas / Q000453:epidemiology; D000075202:Contraindications; D004314:Down Syndrome / Q000150:complications / Q000401:mortality / Q000628:therapy; D015199:Extracorporeal Membrane Oxygenation / Q000009:adverse effects / Q000401:mortality; D005260:Female; D030342:Genetic Diseases, Inborn / Q000150:complications* / Q000401:mortality / Q000628:therapy*; D006331:Heart Diseases / Q000150:complications* / Q000401:mortality / Q000628:therapy*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012189:Retrospective Studies; D013577:Syndrome