Background: As patients began to survive for longer periods following modif
ied Fontan operations (conventional atrio-pulmonary connection), the late m
orbidity after this procedure became increasingly apparent. The purpose of
the present study was to evaluate late sequelae of modified Fontan operatio
ns in long-term survivors (n=14) at our institute, Methods and results: The
cohort consisted of patients who underwent a modified Fontan operation bet
ween 1981 and 1990. Thus. all patients were examined at least 10 years post
operatively in this study. Early mortality, within 30 days of the operation
, was 17.6% (three of 17 patients died from low output syndrome). Excluding
these early deaths, the cumulative survival rate at 5 and 10 pears was 100
and 79%, respectively. Arrhythmias including atrial fibrillation or flutte
r were the main late causes of morbidity. The arrhythmia-free rate at 5 and
10 years was 77 and 50%. respectively, Although the quality of life was co
nsidered good because all patients (n=11) who survived for 10 years or more
were in class I or II according to the New York Heart Association classifi
cation, most of them in fact suffered from potentially life-threatening arr
hythmias. Conclusions. Meticulous attention to and utilization of establish
ed treatment strategies for arrhythmias including anti-arrhythmics, anticoa
gulants. catheter ablation or re-operation converting the circulation to th
e total cavopulmonary connection must be considered in long-term survivors
following the modified Fontan operation. The fact that no one knows when th
e thrombogenic arrhythmias occur suggests anticoagulants should be initiate
d in the early postoperative period. (C) 2001 Elsevier Science Ireland Ltd.
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