Surgical Technique
Re-sternotomy was performed in all cases. The femoral artery and vein
were prepared in cases where the mediastinal structures were adhered to
the sternum (determined with lateral chest x-ray or computed tomography
in the preoperative evaluation), whereas femoral cannulation and
initiation of cardiopulmonary bypass before sternal entrance was not
necessary in any case. Following aortic and bicaval (or tricaval in
cases with a persistent superior venae cavae), aorta was cross clamped
and antegrade blood cardioplegia was administered where intermittent
doses were repeated at every 15 minutes. Oblique right atriotomy was
performed at the lower half of the atrial wall. Pulmonary arteries were
divided above the pulmonary valve except for the cases with pulmonary
atresia. Pulmonary valves were either resected or squeezed within the
polypropylene double row suture line that will close the pulmonary
outflow of the ventricle. If necessary, AV valves were repaired at this
stage of the operation. The PTFE conduit with proper size was
anastomosed to the orifice of IVC in a beveled end-to-end fashion,
carefully observing any accessory hepatic venous drainage to the right
atrium. In patients no. 7 and 17, at least one hepatic vein was
separately draining into right atrium, which were included into Fontan
circulation with the conduit involving their ostium. Afterwards, an
aortic punch was used to fenestrate the conduit at the posterior side,
just above the IVC anastomosis. Then the graft was gently pulled outside
the atrium in order to provide a straight and unkinked part to be
secured inside the atrial cavity. The right atrial incision was closed
with continous polypropylene suture that will enwrap the conduit at the
point where it will be directed out to the pericardial cavity. The
pulmonary arterial anastomosis of the graft was performed with
polypropylene or PTFE sutures. In cases where a pulmonary arterial
augmentation was deemed necessary, a bovine pericardial patch (pt. no
12) or a PTFE patch (pt. no 15) was used. There was no chance to prepare
autologous pericardium for this purpose due to mediastinal adhesions in
these redo cases. Total circulatory arrest was not used in any case. In
patient 12, a MAPCA was occluded with percutaneous intervention one day
before the operation. This patient was the only case where a
cavopulmonary anastomosis and IECF were performed at the same stage.
Temporary inotropic agents were infused in pt. no 12, 13 and 17.
Permanent pace maker implantation was not deemed mandatory in any case.
The mean cardiopulmonary bypass and aortic cross clamp times were 93.1 ±
34.2 and 70.2 ± 23.9 minutes, respectively. All of the patients were
extubated after 7.2 ± 2.6 hours at the intensive care unit (ICU). The
mean ICU stay was 1.7 ± 0.9 days. The chest tubes were kept in place
until the daily drainage was less than 2cc/kg. Mean duration of drainage
was 5.4 ± 2.3 days. Oral warfarin and aspirin were routinely
administered in all of the patients. Steroids were administered when the
daily drainage exceeded 5cc/kg after 5 days of follow up. The target
prothrombin time (international normalized ratio, INR) was 2 to 2.5. We
did not make any second intervention for accumulation of pleural fluid
after the tubes were removed, except for patient no.15, whom we had to
insert pericardial and pleural drainage tubes via subxiphoid approach 13
days after IECF. We did not encounter any phrenic nerve paralysis in
this patient population.
Mean follow up period was 3.6 ± 1.9 years (range: 1 to 7 years). The
mean systemic arterial saturation level at room air was 91.3 ± 2.7 %
(range: 89% to 96%) at the follow up. In pt. no 11, fenestration was
closed via femoral access one year after the IECF. Except this case, all
of the fenestrations are functioning during the follow-up period,
providing a right to left shunt with 5.2 ± 1.3 mmHg shunt gradient
calculated at transthoracic echocardiography. The patients were
discharged with diuretics and angiotensin converting enzyme inhibitors
(when necessary) in order to achieve systemic decongestion and normal
blood pressure.
Fontan failure, associated clinical scenarios (plastic bronchitis or
protein losing enteropathy) and mortality was not encountered in our
patient population.