LONGTERM
FOLLOW-UP AFTER FONTAN OPERATION FOR TRICUSPID ATRESIA: THE DUTCH
EXPERIENCE
Strengers J1, Ottenkamp J2,
Cromme-Dijkhuis A3, Hoorntje T4,
Tanke R5, Veeger N1
University Hospitals of Groningen1, Leiden2,
Rotterdam3, Utrecht4, Nijmegen5, The Netherlands
Introduction: In
this study the long term follow-up is described from all Dutch patients
born with tricuspid atresia who underwent a Fontan operation between 1972
and 1990. Factors, related to morbidity and mortality, are discussed
Material and
Methodes: In this retrospective study 86 patients (41m., 45f.) with
tricuspid atresia were included, who underwent a Fontan operation between
1972 up to 1990. Age at operation varied from 6 month to 23 years, with a
mean of 8.2 years. One patient was lost to follow-up. 29 patients died.
Mean follow-up of the survivors was 18.5 years.
Results: Twenty
years survival is 66%. In one centre there was considerable early mortality
(44%), in the remaining the mortality gradually increased to more than 30%.
Early mortality was characterized by pulmonary hypertension (60%), inter-
mediate and late mortality by rhythm disturbances (50-65%) and also by
chronic heart failure. Morbidity consists of rythm disturbances (47%) and
reoperation (38%),. Only four patients had protein loosing enteropathy.
Conclusion: In this cohort of early Fontan operations pulmonary
hypertension counted for most of the early morality. As rhythm disturbances count for
most of the late mortality and morbidity, close surveillance of the rhythm and
of compromised haemodynamics, leading to rhythm disturbances, might give
the clue to a further reduction in mortality and morbidity.