title: Medium and long-term outcomes of Fontan operation.
abstract: BACKGROUND
  The Fontan operation had been proposed as the final palliative surgery in the patients with single ventricle physiology. Even though modifications of the operation were developed to improve outcomes, long-term complications remain significant with time. The present study reviewed long-term survival rate, morbidities associated with time, and risk factors during the follow-up period after Fontan operation.
  
  
  METHODS
  A retrospective study was conducted. Every patient who underwent the Fontan operation at Siriraj Hospital between January 1987 and December 2007 and had available data was included in the present study. The data was collected until the most recent follow-up in December 2008. Demographic data, diagnosis, echocardiographic data, cardiac catheterization data, surgical data, type of modified Fontan procedure, and perioperative data were collected. The follow-up clinical data, cardiac investigation data, complications, and management were also collected and analyzed.
  
  
  RESULTS
  Survival rates were 88.7%, 85.3%, and 83.8% at 1 year, 5 years, and 10 years, respectively. The median follow-up time was 4.75 years (0-17.45). The 10-years survival rate of tricuspid atresia, single ventricle and the heterotaxy syndrome were 94.5%, 79%, and 83.3%, respectively, which were not significantly different (p = 0.09). The 10-years survival rates of the patients that underwent lateral tunnel, extracardiac conduit and atriopulmonary connection were 80.7%, 88% and 84.3%, respectively. A mean pulmonary artery pressure of more than 18 mmHg was the only factor that affected the survival rate after Fontan surgery (p = 0.008). The incidence of postoperative arrhythmia was 7.9%. Age at operation, diagnosis, type of operation, fenestration, systemic EDP, or PVR before operation did not significantly affect the survival rate. Diagnosis and type of surgery did not affect long-term outcome regarding arrhythmia, re-intervention, systemic atrioventricular valve regurgitation, and systemic ventricular dysfunction. Patients post Fontan operation had good survival rate.
  
  
  CONCLUSIONS
  Cardiac diagnoses were not significantly different in the medium and long-term survival rate of post Fontan patients, freedom from arrhythmia, re-intervention and systemic atrioventricular regurgitation. Types of Fontan operation did not affect long-term survival rate or long-term complications. Mean pulmonary artery pressure of more than 18 mmHg was the only risk factor to the survival rate.
authors.affiliation: Division of Pediatric Cardiology, Department of Pediatrics, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand.
authors.forename: Paweena
authors.initials: P
authors.lastname: Chungsomprasong
authors.forename: Jarupim
authors.initials: J
authors.lastname: Soongswang
authors.forename: Apichart
authors.initials: A
authors.lastname: Nana
authors.forename: Kritvikrom
authors.initials: K
authors.lastname: Durongpisitkul
authors.forename: Daungmanee
authors.initials: D
authors.lastname: Loahaprasitiporn
authors.forename: Chodchanok
authors.initials: C
authors.lastname: Vijansorn
authors.forename: Somchai
authors.initials: S
authors.lastname: Sriyodchartti
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000379:methods*; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D006352:Heart Ventricles / Q000002:abnormalities* / Q000503:physiopathology / Q000601:surgery; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008297:Male; D009017:Morbidity; D010166:Palliative Care; D012189:Retrospective Studies; D012307:Risk Factors; D011795:Surveys and Questionnaires; D015996:Survival Rate; D013785:Thailand / Q000453:epidemiology; D013997:Time Factors; D016896:Treatment Outcome; D016276:Ventricular Function / Q000502:physiology; D055815:Young Adult