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Original Articles: Pediatric Cardiac

How Good Is a Good Fontan? Quality of Life and Exercise Capacity of Fontans Without Arrhythmias

^a Department of Cardiac Surgery, Royal Children's Hospital, Department of Pediatrics of the University of Melbourne, Murdoch Children's Research Institute, and the ANZCHRC, Parkville, Victoria, Australia
^b Department of Cardiology, Royal Children's Hospital, Department of Pediatrics of the University of Melbourne, Murdoch Children's Research Institute, and the ANZCHRC, Parkville, Victoria, Australia
^c Department of Academic Child Psychiatry Unit, Royal Children's Hospital, Department of Pediatrics of the University of Melbourne, Murdoch Children's Research Institute, and the ANZCHRC, Parkville, Victoria, Australia
^d Department of Cardiology, Royal Melbourne Hospital, Parkville, Victoria, Australia

Accepted for publication July 31, 2009.

^_* Address correspondence to Dr d'Udekem, Department of Cardiac Surgery, Royal Children's Hospital, Flemington Rd, Parkville, Melbourne, Victoria, 3052, Australia (Email: yves.dudekem{at}rch.org.au).

Background: Poor long-term outcomes are expected after Fontan surgery, but these perspectives have been tainted by the poorly functioning Fontans suffering from arrhythmias. No predictions of outcome can be quoted to the increasing number of Fontan patients free from arrhythmic complications. The parameters determining improved exercise capacity and quality of life in this subgroup are yet unknown.

Methods: Fontan survivors from our institution and living in Victoria were invited to participate in the study if they were more than 10 years of age, and free of arrhythmias. A mean of 17 ± 4 years after Fontan, 36 patients, 23 with a classical atriopulmonary connection (AP) and 13 with a lateral tunnel (LT) underwent transthoracic echocardiography, cycloergometer exercise study, neurohumoral screening, and assessment of quality of life.

Results: The only factor predicting worse exercise capacity was the type of Fontan performed; patients with LT having better exercise capacity than those with AP (percentage of predicted anaerobic threshold: 88 ± 14% vs 72 ± 14%, p < 0.005; percentage of predicted VO ₂max: 62 ± 8% vs 54 ± 7%, p < 0.005). Endothelin-1 levels were elevated in all patients (2.9 pmol/L, 2.5 to 3.7). Responses from the quality of life measures placed our Fontan cohort mainly within the normal population range. None of the preoperative and postoperative variables adversely affected patients' quality of life.

Conclusions: The anaerobic threshold of arrhythmia-free Fontan patients operated with the lateral tunnel technique was relatively preserved. Despite restricted exercise capacity, Fontan patients, provided that they are free of arrhythmias, have a normal quality of life reflected in their reports of psychiatric symptoms and family relationships.