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(Circulation. 2008;117:85-92.)
© 2008 American Heart Association, Inc.

Pediatric Cardiology

Long-Term Survival, Modes of Death, and Predictors of Mortality in Patients With Fontan Surgery

Paul Khairy, MD, PhD; Susan M. Fernandes, MHP, PA-C; John E. Mayer, Jr, MD; John K. Triedman, MD; Edward P. Walsh, MD; James E. Lock, MD; Michael J. Landzberg, MD

From the Boston Adult Congenital Heart Service, Brigham and Women’s Hospital, Beth Israel Deaconess Medical Center, and Children’s Hospital Boston, Harvard Medical School, Boston, Mass.

Correspondence to Dr Paul Khairy, Adult Congenital Heart Center, Montreal Heart Institute, 5000 Bélanger St, Montreal, Quebec, Canada H1T 1C8 (E-mail paul.khairy{at}cardio.CHBoston.org). Reprint requests to Dr Michael J. Landzberg, Director, Boston Adult Congenital Heart Service, Department of Cardiology, Children’s Hospital Boston, 300 Longwood Ave, Boston, MA 02115 (E-mail michael.landzberg@cardio.CHBoston.org).

Received September 4, 2007; accepted October 22, 2007.

Background— To better define determinants of mortality in patients with univentricular physiology, a database registry was created of patients born in 1985 or earlier with Fontan surgery who were followed up at Children’s Hospital Boston.

Methods and Results— A total of 261 patients, 121 of whom (46.4%) were women, had a first Fontan surgery at a median age of 7.9 years: right atrium–to–pulmonary artery connection in 135 (51.7%); right atrium to right ventricle in 25 (9.6%); and total cavopulmonary connection in 101 (38.7%). Over a median of 12.2 years, 76 (29.1%) died, 5 (1.9%) had cardiac transplantation, 5 (1.9%) had Fontan revision, and 21 (8.0%) had Fontan conversion. Perioperative mortality decreased steadily over time and accounted for 68.4% of all deaths. In early survivors, actuarial freedom from death or transplantation was 93.7%, 89.9%, 87.3%, and 82.6% at 5, 10, 15, and 20 years, respectively, with no significant difference between right atrium to pulmonary artery versus total cavopulmonary connection. Late deaths were classified as sudden in 7 patients (9.2%), thromboembolic in 6 (7.9%), heart failure–related in 5 (6.7%), sepsis in 2 (2.6%), and other in 4 (5.2%). Most sudden deaths were of presumed arrhythmic origin with no identifiable predictor. Independent risk factors for thromboembolic death were lack of antiplatelet or anticoagulant therapy (hazard ratio [HR], 91.6; P=0.0041) and clinically diagnosed intracardiac thrombus (HR, 22.7; P=0.0002). Independent predictors of heart failure death were protein-losing enteropathy (HR, 7.1; P=0.0043), single morphologically right ventricle (HR, 10.5; P=0.0429), and higher right atrial pressure (HR, 1.3 per 1 mm Hg; P=0.0016).

Conclusion— In perioperative survivors of Fontan surgery, gradual attrition occurs predominantly from thromboembolic, heart failure–related, and sudden deaths.

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CLINICAL PERSPECTIVE

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Clinical Summaries
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