title: Revision of previous Fontan connections to total extracardiac cavopulmonary anastomosis: A multicenter experience.
abstract: BACKGROUND
  Conversion to total extracardiac cavopulmonary anastomosis is an option for managing patients with dysfunction of a prior Fontan connection.
  
  
  METHODS
  Thirty-one patients (19.9 +/- 8.8 years) underwent revision of a previous Fontan connection to total extracardiac cavopulmonary anastomosis at four institutions. Complications of the previous Fontan connection included atrial tachyarrhythmias (n = 20), progressive heart failure (n = 17), Fontan pathway obstruction (n = 10), effusions (n = 10), pulmonary venous obstruction by an enlarged right atrium (n = 6), protein-losing enteropathy (n = 3), right atrial thrombus (n = 2), subaortic stenosis (n = 1), atrioventricular valve regurgitation (n = 3), and Fontan baffle leak (n = 5). Conversion to an extracardiac cavopulmonary connection was performed with a nonvalved conduit from the inferior vena cava to the right pulmonary artery, with additional procedures as necessary.
  
  
  RESULTS
  There have been 3 deaths. Two patients died in the perioperative period of heart failure and massive effusions. The third patient died suddenly 8 months after the operation. All surviving patients were in New York Heart Association class I (n = 20) or II (n = 7), except for 1 patient who underwent heart transplantation. Early postoperative arrhythmias occurred in 10 patients: 4 required pacemakers, and medical therapy was sufficient in 6. In 15 patients, pre-revision arrhythmias were improved. Effusions resolved in all but 1 of the patients in whom they were present before revision. The condition of 2 patients with protein-losing enteropathy improved within 30 days.
  
  
  CONCLUSIONS
  Conversion of a failing Fontan connection to extracardiac cavopulmonary connection can be achieved with low morbidity and mortality. Optimally, revision should be undertaken early in symptomatic patients before irreversible ventricular failure ensues.
authors.affiliation: Hesperia Hospital, Modena, Italy.
authors.forename: C F
authors.initials: CF
authors.lastname: Marcelletti
authors.forename: F L
authors.initials: FL
authors.lastname: Hanley
authors.forename: C
authors.initials: C
authors.lastname: Mavroudis
authors.forename: D B
authors.initials: DB
authors.lastname: McElhinney
authors.forename: R F
authors.initials: RF
authors.lastname: Abella
authors.forename: S M
authors.initials: SM
authors.lastname: Marianeschi
authors.forename: F
authors.initials: F
authors.lastname: Seddio
authors.forename: V M
authors.initials: VM
authors.lastname: Reddy
authors.forename: E
authors.initials: E
authors.lastname: Petrossian
authors.forename: T
authors.initials: T
authors.lastname: de la Torre
authors.forename: L
authors.initials: L
authors.lastname: Colagrande
authors.forename: C L
authors.initials: CL
authors.lastname: Backer
authors.forename: A
authors.initials: A
authors.lastname: Cipriani
authors.forename: F S
authors.initials: FS
authors.lastname: Iorio
authors.forename: F
authors.initials: F
authors.lastname: Fontan
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D003430:Cross-Sectional Studies; D018729:Fontan Procedure / Q000401:mortality; D018610:Heart Bypass, Right; D006330:Heart Defects, Congenital / Q000601:surgery*; D006801:Humans; D011183:Postoperative Complications / Q000401:mortality / Q000601:surgery*; D012086:Reoperation / Q000401:mortality; D012189:Retrospective Studies; D015996:Survival Rate; D017211:Treatment Failure