BACKGROUND: Poor long-term outcomes are expected after Fontan surgery, but these perspectives have been tainted by the poorly functioning Fontans suffering from @entity943 . No predictions of outcome can be quoted to the increasing number of Fontan @entity1 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 @entity943 . A mean of 17 +/- 4 years after Fontan, 36 @entity1 , 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; @entity1 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(2)max: 62 +/- 8% vs 54 +/- 7%, p < 0.005). @entity2232 levels were elevated in all @entity1 (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 @entity1 ' quality of life. CONCLUSIONS: The anaerobic threshold of @entity943 -free Fontan @entity1 operated with the lateral tunnel technique was relatively preserved. Despite restricted exercise capacity, Fontan @entity1 , provided that they are free of @entity943 , have a normal quality of life reflected in their reports of @entity146 symptoms and family relationships.

How good is a good Fontan? Quality of life and exercise capacity of Fontans without XXXX .

@entity1 :: ('9606', 'Species') :: ['patients'] @entity943 :: ('MESH:D001145', 'Disease') :: ['arrhythmias', 'arrhythmia'] @entity146 :: ('MESH:D001523', 'Disease') :: ['psychiatric'] @entity2232 :: ('1906', 'Gene') :: ['Endothelin-1']
@entity943 :: (MESH:D001145,Disease) :: ['arrhythmias']
