Surgical Technique
Re-sternotomy was performed in all cases. Femoral cannulation and
initiation of cardiopulmonary bypass before sternal re-entrance was not
deemed mandatory in any of the cases. Following aortic and bicaval (or
tricaval in cases with a persistent superior venae cavae) cannulation
aorta was cross clamped except pt. no 12. Cold antegrade blood
cardioplegia was administered where intermittent doses were applied at
every 15 minutes. Pulmonary artery was divided above the level of
pulmonary valve, except for the cases with pulmonary atresia where there
was no antegrade pulmonary blood flow. Pulmonary cusps were excised or
squeezed between the double row matress suture lines closing the
pulmonary outflow of the ventricle. Inferior venae cavae (IVC) was
transected at the level of atrial entrance and a PTFE tubular conduit
was anastomosed to IVC with an end to end fashion. End to side
anastomosis was performed between the pulmonary artery and the conduit
and care was taken in order not to compress the pulmonary venous return
throughout the course of the conduit from IVC to pulmonary artery in the
pericardial cavity. In cases where an intra-extracardiac technique was
implemented, the anastomosis was performed within the atrium encircling
the ostium of the IVC. Then the conduit was passed from the atrium to
outside into the pericardial cavity. Total circulatory arrest was not
used in any case. When fenestration was needed, in cases with an
extracardiac technique, a side to side anastomosis was performed between
the conduit and the atrium. In intra-extracardiac Fontan cases, a punch
was used to make a fenestration and leave it inside the atrium.
Temporary inotropic agents were infused in pt. no 3,4 and 7 where
dobutamine was the first line choice. In patient no. 14, pulmonary
reconstruction was performed following transection of the ascending
aorta in order to provide better exposure of the pulmonary arteries.
Permanent pace maker was not necessary in any case. The mean
cardiopulmonary bypass and aortic cross clamp times were 69.7 ± 27.1 and
51.6 ± 25.3 minutes, respectively. All of the patients were
hemodynamically stable with the sternum closed when they leaved the
operating room. Any postoperative revision for bleeding or any other
reason was not encountered in the early postoperative period.
The mean duration of intubation and intensive care unit stay were 7.8 ±
3.5 hours and 2.3 ± 3.5 days, respectively. The chest drainage tubes
were in place until the daily drainage was less than 2cc/kg. Mean
duration of drainage was 3.9 ± 5.3 days. Oral warfarin, aspirin,
sildenafil citrate and bosentan were routinely administered in all of
the patients after FP. The target INR was 2 to 2.5. Steroids were
administered when the daily drainage exceeded 5cc/kg. Following removal
of the chest tubes, daily consecutive chest x-rays were obtained in
order to detect pleural effusion. Any chest tube re-insertion was not
needed for re-accumulation of the pleural fluid. We did not encounter
any phrenic nerve paralysis in this patient population. Mean duration of
discharge was recorded as 10 ± 1.8 days.
Mean follow up period was 4.8 ± 7.7 years (range: 1 to 11 years). All of
the patients are on lifelong Aspirin (5mgr/kg) therapy. Oral warfarin
and sildenafil citrate (3mgr/kg in 3 divided doses) are administered at
least for one year, and afterwards individual decision is given
primarily according to a presence of fenestration. Oral endothelin
receptor antagonist, bosentan is administered at least for one month.
All of the patients were discharged with diuretics and angiotensin
converting enzyme inhibitors in order to achieve a normal blood
pressure.
Early or mid-term Fontan failure and mortality was not encountered. In
pt. no 14, fenestration was closed via femoral access one year after the
FP. We have not encountered any case with significant arrhythmia,
plastic bronchitis or protein losing enteropathy in our patient
population. In cases with AV valve repair, trace to mild regurgitations
are encountered. All of the patients in our population are followed up
with ejection fractions above 50% with NYHA class I-II symptoms at
annual outpatient visits.