Permanent pacing is often required following the Fontan operation and
is usually performed epicardially as there is no direct access to the
ventricle from the systemic veins. Dual chamber endocardial pacing was
achieved by the transhepatic approach in two children with Fontan cir
culation. The patients were a 7 year old boy with left atrial isomeris
m, single ventricle with pulmonary stenosis, interrupted inferior vena
caval vein with azygous continuation, and direct drainage of the hepa
tic veins to the right sided atrium, and a 6 year old girl with tricus
pid atresia. This approach to endocardial pacemaker implantation is po
tentially of considerable value in patients who do not have direct acc
ess to the ventricle from the systemic veins.