Long-term survival after the Fontan operation: Twenty years of experience at a single center.
Existing studies of patients palliated with the Fontan operation are limited by heterogeneous patient populations and incomplete follow-up. This study aimed to describe long-term post-Fontan survival in a modern patient cohort.



All 773 patients who underwent a first Fontan operation at our institution between 1992 and 2009 were reviewed. The primary outcome was the composite endpoint of Fontan takedown, heart transplantation, or death before 2013.



Follow-up rate was 99.2%. Survival with intact Fontan circulation was 94% at 1 year (95% confidence interval [95% CI], 92%-95%), 90% at 10 years (95% CI, 88%-92%), 85% at 15 years (95% CI, 82%-88%), and 74% at 20 years (95% CI, 67%-80%). Distinct risk factors were identified for early (≤1 year) and late composite outcomes. Independent risk factors for early outcome included prolonged pleural drainage (hazard ratio [HR], 4.4; P < .001), intensive care unit stay >1 week (HR, 2.4; P < .001), Fontan before 1997 (HR, 3.3; P < .001), preoperative atrioventricular valve regurgitation (HR, 2.0; P < .001), and longer crossclamp time (HR, 1.3 per 10 minutes; P < .001). Late outcome was predicted by atrioventricular valve regurgitation prior to Fontan (HR, 2.0; P ≤ .001), and post-Fontan ICU stay >1 week (HR, 2.4; P < .001).



Long-term mortality after Fontan operation remains substantial. Risk factors for death or loss of Fontan circulation differ between the early and late postoperative periods. Long-term survival has not improved appreciably over the last decade, suggesting that alternatives to the Fontan are warranted.
Division of Pediatric Cardiology, The Cardiac Center at The Children's Hospital of Philadelphia, Philadelphia, Pa.
Tacy E
TE
Downing
Division of Pediatric Cardiology, The Cardiac Center at The Children's Hospital of Philadelphia, Philadelphia, Pa.
Kiona Y
KY
Allen
Division of Pediatric Cardiology, The Cardiac Center at The Children's Hospital of Philadelphia, Philadelphia, Pa.
Andrew C
AC
Glatz
Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
Lindsay S
LS
Rogers
Division of Pediatric Cardiology, The Cardiac Center at The Children's Hospital of Philadelphia, Philadelphia, Pa.
Chitra
C
Ravishankar
Division of Pediatric Cardiology, The Cardiac Center at The Children's Hospital of Philadelphia, Philadelphia, Pa.
Jack
J
Rychik
Department of Pediatrics, The Cardiac Center at The Children's Hospital of Philadelphia, Philadelphia, Pa.
Jennifer A
JA
Faerber
Department of Surgery, The Cardiac Center at The Children's Hospital of Philadelphia, Philadelphia, Pa.
Stephanie
S
Fuller
Department of Anesthesiology and Critical Care, The Cardiac Center at The Children's Hospital of Philadelphia, Philadelphia, Pa.
Lisa M
LM
Montenegro
Department of Anesthesiology and Critical Care, The Cardiac Center at The Children's Hospital of Philadelphia, Philadelphia, Pa.
James M
JM
Steven
Department of Surgery, The Cardiac Center at The Children's Hospital of Philadelphia, Philadelphia, Pa.
Thomas L
TL
Spray
Department of Anesthesiology and Critical Care, The Cardiac Center at The Children's Hospital of Philadelphia, Philadelphia, Pa.
Susan C
SC
Nicolson
Department of Surgery, The Cardiac Center at The Children's Hospital of Philadelphia, Philadelphia, Pa.
J William
JW
Gaynor
Division of Pediatric Cardiology, The Cardiac Center at The Children's Hospital of Philadelphia, Philadelphia, Pa. Electronic address: goldbergda@email.chop.edu.
David J
DJ
Goldberg
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D018729:Fontan Procedure / Q000401:mortality*; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D015996:Survival Rate; D013997:Time Factors; D055815:Young Adult
cardiovascular surgery; congenital heart disease; mortality/survival; transplantation