This Article Full Text Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via Google Scholar Google Scholar Articles by d’Udekem, Y. Articles by Brizard, C. P. Search for Related Content PubMed PubMed Citation Articles by d’Udekem, Y. Articles by Brizard, C. P. Related Collections Arrhythmias, clinical electrophysiology, drugs Pediatric and congenital heart disease, including cardiovascular surgery

(Circulation. 2007;116:I-157 – I-164.)
© 2007 American Heart Association, Inc.

Surgery for Congenital Heart Disease

The Fontan Procedure

Contemporary Techniques Have Improved Long-Term Outcomes

Yves d’Udekem, MD, PhD; Ajay J. Iyengar, BmedSci; Andrew D. Cochrane, MD, FRACS; Leeanne E. Grigg, MBBS, FRACP; James M. Ramsay, MD, FRACP; Gavin R. Wheaton, MD, FRACP; Dan J. Penny, MD, PhD, FRACP; Christian P. Brizard, MD

From the Departments of Cardiac Surgery (Y.d’U., A.J.I., A.D.C., C.P.B.) and Cardiology (D.J.P.), Royal Children’s Hospital and the Department of Pediatrics, the University of Melbourne; Department of Cardiology (L.G.), Royal Melbourne Hospital; Department of Cardiology (J.M.R.), Princess Margaret Hospital for Children, Perth; Department of Cardiology (G.R.W.), Adelaide Women’s and Children’s Hospital, Adelaide; Australia and New Zealand Children’s Heart Research Center, Australia.

Correspondence to Yves d’Udekem, Department of Cardiac Surgery, Royal Children’s Hopital, Flemington Road, Parkville, Melbourne 3052, Victoria Australia. E-mail yves.dudekem{at}rch.org.au

Background— To determine whether patients undergoing the lateral tunnel and extracardiac conduit modifications of the Fontan procedure have better outcomes than patients undergoing a classical atriopulmonary connection.

Methods and Results— Between 1980 and 2000, 305 consecutive patients underwent a Fontan procedure at our institution. There were 10 hospital deaths (mortality: 3%) with no death after 1990. Independent risk factors for mortality were preoperative elevated pulmonary artery pressures (P=0.002) and common atrioventricular valve (P=0.04). Fontan was taken down during hospital stay in 7 patients. A mean of 12±6 years of follow-up was obtained in the 257 nonforeign Fontan survivors. Completeness of concurrent follow-up was 96%. Twenty-year survival was 84% (95% CI: 79 to 89%). Recent techniques improved late survival. The 15-year survival after atriopulmonary connection was 81% (95% CI: 73% to 87%) versus 94% (95% CI: 79% to 98%) for lateral tunnel (P=0.004). Nine pts required heart transplantation (8 atriopulmonary connection, 1 lateral tunnel). Undergoing a Fontan modification independently predicted decreased occurrence of arrhythmia, and 15-year freedom from SVT was 61% (95% CI: 51% to 70%) for atriopulmonary connection versus 87% (95% CI: 76% to 93%) for lateral tunnel (P=0.02). Freedom from Fontan failure (death, take-down, transplantation, or NYHA class III-IV) was 70% (95% CI: 58% to 79%) at 20 years. After extra-cardiac conduits, no death, SVT, or failure was observed.

Conclusions— The Fontan procedure remains a palliation, but outcomes of patients have improved. Better patient selection minimizes hospital mortality. Patients with lateral tunnel and extracardiac conduit modifications experience less arrhythmia and are likely to have failure of their Fontan circulation postponed.

Key Words: Fontan procedure • follow-up studies • pediatrics