149 Fontan Conversions

Methodist Debakey Cardiovasc J. 2019 Apr-Jun;15(2):105-110. doi: 10.14797/mdcj-15-2-105.


Patients with a functionally univentricular heart who have had an atriopulmonary Fontan are at risk for atrial dilatation, atrial arrhythmias, and progressive circulatory failure. Between 1994 and 2018, we performed 149 Fontan conversions with arrhythmia surgery and epicardial pacemaker placement at Ann & Robert H. Lurie Children's Hospital of Chicago. This operation converts the atriopulmonary Fontan to an extracardiac Fontan that improves hemodynamics and controls the atrial arrhythmias. Operative mortality during that time was 2%, and freedom from death or heart transplant at 10 years is 84%. For properly selected patients, Fontan conversion improves both survival and quality of life. Patients with an atriopulmonary Fontan constitute an eroding population, as they face many comorbidities and have a decreased life expectancy without treatment; therefore, all patients with an atriopulmonary Fontan should be evaluated for this procedure.

Keywords: Fontan conversion; arrhythmia; atrial fibrillation; atriopulmonary Fontan; congenital heart surgery; cryoablation; epicardial pacemaker; protein-losing enteropathy; tricuspid atresia.

MeSH terms

  • Adolescent
  • Adult
  • Arrhythmias, Cardiac / etiology
  • Arrhythmias, Cardiac / mortality
  • Arrhythmias, Cardiac / physiopathology
  • Arrhythmias, Cardiac / surgery*
  • Cardiomegaly / etiology
  • Cardiomegaly / mortality
  • Cardiomegaly / physiopathology
  • Cardiomegaly / surgery*
  • Child
  • Child, Preschool
  • Clinical Decision-Making
  • Female
  • Fontan Procedure* / adverse effects
  • Fontan Procedure* / mortality
  • Heart Defects, Congenital / complications
  • Heart Defects, Congenital / mortality
  • Heart Defects, Congenital / physiopathology
  • Heart Defects, Congenital / surgery*
  • Humans
  • Male
  • Middle Aged
  • Patient Selection
  • Progression-Free Survival
  • Reoperation
  • Risk Assessment
  • Risk Factors
  • Time Factors
  • Young Adult