Long-Term Outcome and Quality of Life in Adult Patients After the Fontan Operation Annemien E. van den Bosch, MD, Jolien W. Roos-Hesselink, MD, Ron van Domburg, PhD, Ad J.J.C. Bogers, MD, PhD, Maarten L. Simoons, MD, PhD, and Folkert J. Meijboom, MD, PhD The first successful Fontan operation was performed in 1971, and this first cohort of Fontan patients is reaching adulthood with unclear outcome of this palliative proce- dure. We studied the mortality, morbidity, and quality of life in our adult Fontan patients. We examined all pa- tients (n  36) who underwent a Fontan procedure and were being seen in an adult outpatient clinic by using electrocardiography, exercise testing, and echocardiog- raphy. Quality of life was assessed by the Short Form 36 questionnaire. The mean follow-up period was 15 years (range 0 to 23). Of the initial 36 patients, 10 died (28%) at a mean of 10 years (range 0 to 21) after the Fontan operation and 1 patient underwent cardiac transplanta- tion. Reoperations were performed in 21 patients (58%), and the most common reason was revision of the Fontan connection. Sustained supraventricular tachycardia was observed in 20 patients (56%) with an increased inci- dence of arrhythmias with longer follow-up. Thrombo- embolic events were detected in 9 patients (25%), 5 of whom had adequate anticoagulant levels at the time of event. The thromboembolic event was fatal for 3 pa- tients. A total of 195 hospital admissions (mean 3.8  2.7, range 1 to 13) was recorded. Quality-of-life assess- ment showed physical functioning, mental health, and general health perception to be significantly lower for Fontan patients than for the normal Dutch population. Thus, we found high mortality and very high morbidity in adult patients after the Fontan operation. In particu- lar, reoperations, arrhythmias, and thromboembolic events compromised quality of life. 2004 by Ex- cerpta Medica, Inc. (Am J Cardiol 2004;93:1141–1145) S ince Fontan and Baudet's report of the first suc- cessful right-side cardiac bypass directing the en- tire systemic venous blood flow to the pulmonary arteries in a patient with tricuspid atresia, many mod- ifications of this approach have been applied to all forms of functional univentricular heart disease. Dur- ing the past 2 decades, several modifications of this operation and advances in management after surgery have improved surgical results. 1,2 Unfortunately, late deterioration in functional capacity has been described with longer duration of follow-up. 3 As hospital mor- tality has decreased substantially, late mortality and especially late morbidity are of great interest. 4–6 The occurrence of late complications such as atrial ar- rhythmias, ventricular failure, protein-losing entero- pathy, and thromboembolic events are increasingly recognized. 7–9 No reports are available concerning quality of life in adult patients with a Fontan circula- tion. We evaluated the clinical course of adult Fontan patients and assessed their quality of life. METHODS Patients: All adult patients who underwent a Fontan procedure and regularly attended the outpatient clinic of the Thoraxcenter at the Erasmus Medical Center were included in this study. In 1978, the first Fontan operation was performed in our institute. We studied the long-term follow-up from Fontan operation until last follow-up or death. In 2002, a cross-sectional study of surviving patients was undertaken. All med- ical and surgical records of the patients were reviewed for reoperations, arrhythmias, hospitalization, and thromboembolic events. The cross-sectional evalua- tion consisted of physical examination, electrocardi- ography, exercise testing, and echocardiography. Quality of life was assessed with the Short Form 36 (SF-36) questionnaire. Arrhythmias: The presence of an arrhythmia on any recording device was sufficient to code a patient for that rhythm disturbance, excluding arrhythmias re- lated to cardiac catheterization or the period after surgery. Supraventricular arrhythmia included any sustained episode of atrial flutter, atrial fibrillation, or atrial tachycardia occurring at least 30 days after the Fontan operation. Exercise capacity: Maximal exercise capacity was assessed by bicycle ergometry with stepwise incre- ments of 10 W per minute for workload and compared with standardized data based on age, gender, and height. Echocardiography: Two-dimensional echocardiog- raphy with color Doppler, velocity profiles, and M- mode recordings were performed. The Fontan connec- tion was evaluated for obstruction in the conduit. Systemic ventricular function was judged by visual From the Departments of Cardiology and Cardiothoracic Surgery, Thoraxcenter, Erasmus MC, Rotterdam, The Netherlands. Manuscript received July 16, 2003; revised manuscript received and accepted January 12, 2004. Address for reprints: Jolien W. Roos-Hesselink, MD, Thoraxcenter Ba 308, Erasmus MC, Dr Molewaterplein 40, 3015 GD Rotterdam, The Netherlands. E-mail: j.roos@erasmusmc.nl. 1141 ©2004 by Excerpta Medica, Inc. All rights reserved. 0002-9149/04/$–see front matter The American Journal of Cardiology Vol. 93 May 1, 2004 doi:10.1016/j.amjcard.2004.01.041