title: 40-Year Follow-Up After the Fontan Operation: Long-Term Outcomes of 1,052 Patients.
abstract: BACKGROUND
  There are limited long-term, single-cohort, follow-up studies available about patients after the Fontan operation.
  
  
  OBJECTIVE
  This study sought to determine the long-term outcome of all patients who had a Fontan operation at the Mayo Clinic.
  
  
  METHODS
  Records of all patients who had a modified Fontan operation between 1973 and 2012 were reviewed. A follow-up questionnaire was mailed to all patients alive at the time of the study.
  
  
  RESULTS
  Overall, 10-, 20-, and 30-year survival for 1,052 patients was 74%, 61%, and 43%, respectively. Factors associated with decreased overall or late survival in multivariate analysis included pre-operative diuretic use, longer cardiopulmonary bypass time, operation prior to 1991, atrioventricular valve (AVV) replacement at the time of Fontan operation, elevated post-bypass Fontan (>20 mm Hg) or left atrial (>13 mm Hg) pressures, prolonged chest tube drainage (>21 days), post-operative ventricular arrhythmias, renal insufficiency, and development of protein-losing enteropathy (PLE). Pre-operative and intraoperative sinus rhythm were associated with improved survival. Long-term survival was similar for patients regardless of ventricular morphology. The most common reoperations were pacemaker insertion/revision in 212 patients (20%), Fontan revision/conversion in 117 patients (11%), and AVV repair/replacement in 66 patients (5%). Clinically significant late atrial or ventricular arrhythmias occurred in 468 patients (44%). Ninety-five patients (9%) developed PLE, and 5-, 10-, and 20-year survival after diagnosis of PLE was 50%, 35%, and 19%, respectively.
  
  
  CONCLUSIONS
  As the surgical techniques for the Fontan operation have changed over the last 40 years, survival has improved. However, development of PLE and arrhythmias and the need for reoperation during long-term follow-up pose significant management challenges.
authors.affiliation: Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota.
authors.forename: Kavitha N
authors.initials: KN
authors.lastname: Pundi
authors.affiliation: Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota; Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota.
authors.forename: Jonathan N
authors.initials: JN
authors.lastname: Johnson
authors.affiliation: Division of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota.
authors.forename: Joseph A
authors.initials: JA
authors.lastname: Dearani
authors.affiliation: Mayo Clinic College of Medicine, Mayo Clinic, Rochester, Minnesota.
authors.forename: Krishna N
authors.initials: KN
authors.lastname: Pundi
authors.affiliation: Division of Biomedical Statistics and Informatics, Mayo Clinic, Rochester, Minnesota.
authors.forename: Zhuo
authors.initials: Z
authors.lastname: Li
authors.affiliation: Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota.
authors.forename: Cynthia A
authors.initials: CA
authors.lastname: Hinck
authors.affiliation: Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota.
authors.forename: Sonja H
authors.initials: SH
authors.lastname: Dahl
authors.affiliation: Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota; Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota.
authors.forename: Bryan C
authors.initials: BC
authors.lastname: Cannon
authors.affiliation: Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota; Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota.
authors.forename: Patrick W
authors.initials: PW
authors.lastname: O'Leary
authors.affiliation: Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota.
authors.forename: David J
authors.initials: DJ
authors.lastname: Driscoll
authors.affiliation: Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota; Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota. Electronic address: cetta.frank@mayo.edu.
authors.forename: Frank
authors.initials: F
authors.lastname: Cetta
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D005260:Female; D018729:Fontan Procedure / Q000379:methods*; D005544:Forecasting; D006330:Heart Defects, Congenital / Q000601:surgery*; D006801:Humans; D015994:Incidence; D007223:Infant; D008297:Male; D008875:Middle Aged; D008910:Minnesota / Q000453:epidemiology; D011183:Postoperative Complications / Q000453:epidemiology*; D012086:Reoperation; D012189:Retrospective Studies; D012307:Risk Factors; D011795:Surveys and Questionnaires; D015996:Survival Rate / Q000639:trends; D016896:Treatment Outcome; D055815:Young Adult
keywords: Fontan procedure; protein-losing enteropathy; survival