title: The modified Fontan procedure with use of extracardiac conduit in adults: analysis of 32 consecutive patients.
abstract: BACKGROUND
  Uncommonly, adults with functionally univentricular hearts are becoming candidates for a Fontan procedure. The purpose of this study was to evaluate the course of patients undergoing the modified Fontan procedure with an extracardiac conduit in recent years.
  
  
  METHODS
  Between January 2003 and December 2013, 32 adult patients (17 female and 15 male) underwent total cavopulmonary connection (TCPC) with extracardiac conduit. The median age at procedure was 24.5 years (interquartile range [IQR] 20 to 33 years). The diagnoses included double-inlet left ventricle (DILV) in 10 patients (31.2%), tricuspid atresia in 8 patients (25%), double-outlet right ventricle in 4 patients (12.5%), heterotaxia in 4 patients (12.5%), and mitral atresia in 2 patients (6.2%). Seventy-eight percent of patients had undergone at least one prior palliative procedure; the most common procedures were Blalock-Taussig shunt (16 patients), superior cavopulmonary shunt (12 patients), and pulmonary artery banding (6 patients). All patients underwent cardiac catheterization preoperatively. Aortic cross-clamping was necessary in 15 patients for intracardiac procedures. Fenestration was required in 9 patients (28%). Four concomitant intraoperative cryoablation procedures were performed.
  
  
  RESULTS
  There was no hospital mortality. One patient (3.1%) died 6 months after undergoing TCPC. Morbidities included prolonged pleural effusion lasting more than 7 days in 20 patients (62.5%), atrial arrhythmias in 4 patients (12.5%), and permanent pacemakers in 3 patients (9.3%). The median follow-up time was 33 months (interquartile range [IQR], 10.5 to 50 months). Actuarial survival was 91.83% (95% confidence limits, 71.07 to 97.89) at 1 year and 5 years. Ninety-two percent of patients were in New York Heart Association class I or II at follow-up. The median postoperative oxygen saturation was 95% (IQR, 93% to 95.5%). Cardiac arrhythmia occurred in 4 patients. Systolic ventricular function improved during follow-up for all patients except 1 patient, who underwent cardiac transplantation 7 months after the TCPC.
  
  
  CONCLUSIONS
  The modified Fontan procedure with use of an extracardiac conduit can be performed in adults with encouraging early and midterm results. The majority of late survivors had improved quality of life. The incidence of late death, reoperation, arrhythmias, and thromboembolic events was low during follow-up.
authors.affiliation: Department of Congenital Heart Disease, Marie Lannelongue Hospital/M3C, Paris-Sud University, Le Plessis-Robinson, France. Electronic address: m.ly@ccml.fr.
authors.forename: Mohamedou
authors.initials: M
authors.lastname: Ly
authors.affiliation: Department of Congenital Heart Disease, Marie Lannelongue Hospital/M3C, Paris-Sud University, Le Plessis-Robinson, France.
authors.forename: François
authors.initials: F
authors.lastname: Roubertie
authors.affiliation: Department of Congenital Heart Disease, Marie Lannelongue Hospital/M3C, Paris-Sud University, Le Plessis-Robinson, France.
authors.forename: Reda
authors.initials: R
authors.lastname: Kasdi
authors.affiliation: Department of Congenital Heart Disease, Marie Lannelongue Hospital/M3C, Paris-Sud University, Le Plessis-Robinson, France.
authors.forename: Sana
authors.initials: S
authors.lastname: Chatti
authors.affiliation: Department of Congenital Heart Disease, Marie Lannelongue Hospital/M3C, Paris-Sud University, Le Plessis-Robinson, France.
authors.forename: Mathieu
authors.initials: M
authors.lastname: Vergnat
authors.affiliation: Department of Congenital Heart Disease, Marie Lannelongue Hospital/M3C, Paris-Sud University, Le Plessis-Robinson, France.
authors.forename: David
authors.initials: D
authors.lastname: Luu
authors.affiliation: Department of Congenital Heart Disease, Marie Lannelongue Hospital/M3C, Paris-Sud University, Le Plessis-Robinson, France.
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Le Bret
authors.affiliation: Department of Congenital Heart Disease, Marie Lannelongue Hospital/M3C, Paris-Sud University, Le Plessis-Robinson, France.
authors.forename: Régine
authors.initials: R
authors.lastname: Roussin
authors.affiliation: Faculty of Medicine, Paris-Sud University, Orsay, France.
authors.forename: André
authors.initials: A
authors.lastname: Capderou
authors.affiliation: Department of Congenital Heart Disease, Marie Lannelongue Hospital/M3C, Paris-Sud University, Le Plessis-Robinson, France.
authors.forename: Emre
authors.initials: E
authors.lastname: Belli
mesh_terms: D000328:Adult; D002315:Cardiopulmonary Bypass / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000379:methods*; D018610:Heart Bypass, Right / Q000379:methods; D006330:Heart Defects, Congenital / Q000601:surgery*; D006801:Humans; D008297:Male; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult