Longitudinal Follow-Up of Fontan Patients

Study Questions:

What are the longitudinal outcomes of single ventricle patients after Fontan palliation in a multicenter cohort?


This is a follow-up study of the Fontan Cross-Sectional Study (Fontan 1), which had an original cohort of 546 subjects. Exercise testing, echocardiography, B-type natriuretic peptide (BNP), functional health assessment, and medical history abstraction were repeated 9.4 ± 0.4 years after the original analysis and were compared with previous values. Cox regression analysis explored risk factors for interim death or cardiac transplantation.


From the original cohort of 546 subjects, 466 were re-contacted and 373 (80%) were enrolled at 21.2 ± 3.5 years of age. Since the initial study (Fontan 1), 54 subjects (10%) have received a transplant (n = 23) or died without transplantation (n = 31). Cumulative complications since the Fontan included catheter intervention (62%), additional cardiac surgery (32%), arrhythmia requiring treatment (32%), thrombosis (12%), and protein-losing enteropathy (8%). In paired analysis, percent predicted maximum VO2 decreased (69 ± 14 vs. 61 ± 16, p < 0.001, n = 95), and ejection fraction decreased (58 ± 11 vs. 55 ± 10, p < 0.001, n = 259). At latest follow-up, lower Pediatric Quality of Life Inventory physical summary score was associated with decreased exercise performance (R2 adjusted = 0.20, p < 0.001, n = 274). The interval risk of death/transplantation was not associated with ventricular morphology, subject age, or type of Fontan.


The authors concluded that exercise performance decreased over time in Fontan patients and was associated with worse functional health status.


One of the greatest challenges facing practitioners caring for patients with congenital heart disease is the increasing number of patients reaching adulthood after Fontan palliation in childhood. These data emphasize the palliative nature of the Fontan procedure. Morbidity and need for re-intervention are high, with a majority of patients requiring catheter intervention (although a large number of interventions were likely for fenestration closure). Of concern is the insidious nature of disease progression, with decline in maximum VO2 on exercise testing as well as decline in systemic ventricular ejection fraction. No difference was seen in patients with systemic right ventricles as compared with those with systemic left ventricles. The data offer a role for interventions designed at increasing exercise capacity, such as formal exercise programs, in optimizing the long-term functional status of Fontan patients. Finally, the data suggest the need for a systemic and coordinated approach to management of patients with failing Fontan physiology, particularly in regards to timing of transplant evaluation.

Clinical Topics: Arrhythmias and Clinical EP, Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Invasive Cardiovascular Angiography and Intervention, Noninvasive Imaging, Implantable Devices, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Cardiac Surgery and Arrhythmias, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and Heart Failure, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Imaging, CHD and Pediatrics and Interventions, CHD and Pediatrics and Quality Improvement, Heart Transplant, Interventions and Imaging, Interventions and Structural Heart Disease, Echocardiography/Ultrasound

Keywords: Arrhythmias, Cardiac, Cardiac Surgical Procedures, Child, Disease Progression, Echocardiography, Exercise Test, Fontan Procedure, Heart Defects, Congenital, Heart Transplantation, Natriuretic Peptide, Brain, Pediatrics, Protein-Losing Enteropathies, Risk Factors, Stroke Volume, Thrombosis

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