title: Extracorporeal membrane oxygenation support of the Fontan and bidirectional Glenn circulations.
abstract: BACKGROUND
  Extracorporeal membrane oxygenation can provide effective mechanical circulatory support for the failing circulation in children. Patients with failing Fontan and bidirectional Glenn physiology present additional challenges both for extracorporeal membrane oxygenation cannulation and support. We report our institutional experience in patients with cavopulmonary connections who received extracorporeal membrane oxygenation.
  
  
  METHODS
  We performed a retrospective review of 20 patients with cavopulmonary connections (14 Fontan and 6 bidirectional Glenn) who were supported with extracorporeal membrane oxygenation from a single, large pediatric tertiary care center.
  
  
  RESULTS
  Of the 20 patients, ten were supported and decannulated successfully (50%) (two after cardiac transplantation), but only six (30%) are alive at follow-up. Of the 14 Fontan patients, seven (50%) were withdrawn from extracorporeal membrane oxygenation or died within 48 hours of decannulation due to lack of myocardial recovery or severe neurologic injury. All four adult-sized (> 40 kg) Fontan patients were withdrawn from extracorporeal support. The seven Fontan patients who were successfully decannulated survived to discharge, and five (35.7%) are alive at follow-up (median 35 months; range, 7 to 140 months). Of the six bidirectional Glenn patients, five died before hospital discharge and the lone survivor has neurologic injury at follow-up.
  
  
  CONCLUSIONS
  Patients with failing Fontan and bidirectional Glenn physiology present significant challenges to successful extracorporeal membrane oxygenation support. While the morbidity and mortality rates are high, there are select patients for whom extracorporeal support can be effective and lifesaving as a short-term resuscitative intervention.
authors.affiliation: Department of Pediatrics, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts 02115, USA. karen.booth@cardio.chboston.org
authors.forename: Karen L
authors.initials: KL
authors.lastname: Booth
authors.forename: Stephen J
authors.initials: SJ
authors.lastname: Roth
authors.forename: Ravi R
authors.initials: RR
authors.lastname: Thiagarajan
authors.forename: Melvin C
authors.initials: MC
authors.lastname: Almodovar
authors.forename: Pedro J
authors.initials: PJ
authors.lastname: del Nido
authors.forename: Peter C
authors.initials: PC
authors.lastname: Laussen
mesh_terms: D000293:Adolescent; D000328:Adult; D000714:Anastomosis, Surgical; D002648:Child; D002675:Child, Preschool; D015199:Extracorporeal Membrane Oxygenation / Q000009:adverse effects; D018729:Fontan Procedure; D006801:Humans; D007223:Infant; D010166:Palliative Care; D011183:Postoperative Complications / Q000628:therapy; D011651:Pulmonary Artery / Q000601:surgery*; D012131:Respiratory Insufficiency / Q000209:etiology / Q000628:therapy; D012189:Retrospective Studies; D016896:Treatment Outcome; D014683:Vena Cava, Superior / Q000601:surgery*