title: Clinical outcome of 193 extracardiac Fontan patients: the first 15 years.
abstract: OBJECTIVE
  We sought to evaluate the mid-term outcome of hospital survivors with extracardiac Fontan circulation.
  
  
  BACKGROUND
  Few data exist about the mid-term and long-term results of the extracardiac Fontan operation.
  
  
  METHODS
  From November 1988 to November 2003, 221 patients underwent an extracardiac Fontan procedure as primary (9 patients) or secondary (212 patients) palliation, at a mean age of 72.2 months (range 13.1 to 131.3 months). A total of 165 of 193 early survivors underwent programmed noninvasive follow-up evaluations and at least one cardiac catheterization.
  
  
  RESULTS
  The overall survival, including operative deaths, was 85% at 15 years. Freedom from late failure among hospital survivors is 92% at 15 years. A total of 127 of 165 survivors (77%) were in New York Heart Association functional class I. The incidence of late major problems was 24% (42 major problems in 36 of 165 patients): 19 patients had arrhythmias (11%), 5 patients had obstruction of the extracardiac conduit (3%) and 6 of the left pulmonary artery (3.5%), and 5 patients experienced ventricular failure (3%), leading to heart transplantation in 3 patients. Protein-losing enteropathy was found in two patients (1%). The incidence of late re-interventions was 12.7% (21 of 165 patients, including 15 epicardial pacemaker implantations). Four patients died (2.3%), two after heart transplantation.
  
  
  CONCLUSIONS
  After 15 years of follow-up, the overall survival, the functional status, and the cardiopulmonary performance of survivors of the extracardiac Fontan procedure compare favorably with other series of patients who underwent the lateral tunnel approach. The incidence of late deaths, obstructions of the cavopulmonary pathway, re-interventions, and arrhythmias is lower than that reported late after other Fontan-type operations.
authors.affiliation: Department of Pediatric Cardiology and Cardiac Surgery, Rome, Italy. s.giannico@opbg.net
authors.forename: Salvatore
authors.initials: S
authors.lastname: Giannico
authors.forename: Fatma
authors.initials: F
authors.lastname: Hammad
authors.forename: Antonio
authors.initials: A
authors.lastname: Amodeo
authors.forename: Guido
authors.initials: G
authors.lastname: Michielon
authors.forename: Fabrizio
authors.initials: F
authors.lastname: Drago
authors.forename: Attilio
authors.initials: A
authors.lastname: Turchetta
authors.forename: Roberto
authors.initials: R
authors.lastname: Di Donato
authors.forename: Stephen P
authors.initials: SP
authors.lastname: Sanders
mesh_terms: D002318:Cardiovascular Diseases / Q000209:etiology / Q000601:surgery; D013504:Cardiovascular Surgical Procedures; D002648:Child; D002675:Child, Preschool; D005080:Exercise Test; D017079:Exercise Tolerance; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000009:adverse effects; D006330:Heart Defects, Congenital / Q000601:surgery*; D006801:Humans; D007223:Infant; D011651:Pulmonary Artery / Q000601:surgery; D020127:Recovery of Function; D012086:Reoperation; D012129:Respiratory Function Tests; D016019:Survival Analysis; D016896:Treatment Outcome; D014654:Vascular Patency; D014682:Vena Cava, Inferior / Q000601:surgery