RESULTS
Sixteen patients (94%) had situs solitus and one patient (6%) had
situs inversus. Sixteen patients (94%) were palliated as univentricular
physiology due to the hypoplasia of one of the ventricles. One patient
(pt. no 12) with the diagnosis of DORV, Rastelli type C complete
atrioventricular septal defect (CAVSD) and pulmonary artery hypoplasia
had well developed left and right ventricles. However, the straddling of
the chordae prohibited a biventricular repair. Three patients (17%)
(pt. no 2,8 and 16) had tricuspid atresia (TA) and 2 patients (11%) had
mitral atresia (pt. no 5 and 11). We encountered double inlet ventricles
in 5 cases (29%) (pt. no 3,6,7,13 and 15). Three cases (17%) (pt. no
4,9 and 17) had CAVSD with unbalanced ventricular anatomy where a
univentricular palliation was performed. We did not encounter any case
with significant arrhythmia, except patient no.1, who underwent
successful ablation of the intraatrial reentry tachycardia at the
preoperative period.
Stage-1 palliation was deemed necessary in 10 cases (58%). These
interventions included pulmonary banding (PB) alone (11%) (pt. no 3 and
10), systemic to pulmonary artery shunts alone (11%) (pt. no 6 and 11),
PB with concomitant supramitral ring resection and surgical atrial
septectomy (pt. no 5), shunt along with major aortopulmonary collateral
(MAPCA) banding (pt. no 12), PB with atrial septectomy (pt. no 13), PB
with aortic arch reconstruction and coarctation repair (pt. no 15).
Balloon atrial septostomy and patent ductus arteriosus stenting were
performed in two cases (pt. no 14 and pt. no 16, respectively) via
percutaneous route. In one patient (pt. no12), a second operation for
stage-1 palliation was deemed mandatory in order to augment the right
pulmonary artery blood flow with patch plasty.
Stage-2 palliation included bidirectional cavopulmonary anastomosis and
associated interventions, except for patient no. 12 in whom the
cavopulmonary anastomosis was performed along with intraextracardiac
Fontan (IECF) procedure at stage 3. Systemic-to-pulmonary artery shunts
were closed in two patients (pt. no 6 and 11) in stage 2. Concomitant
procedures included patent ductus arteriosus ligation (pt. no 9),
tricuspid valve repair with PB and pulmonary artery mobilization (pt. no
11), atrial septectomy and PB (pt. no 14), ventricular septal defect
enlargement, subaortic membrane resection and atrial septectomy (pt. no
15) and left pulmonary artery patch augmentation (pt. no 16). In patient
no.16, left pulmonary artery was stented and a left modified
Blalock-Taussig shunt procedure was performed in another cardiac surgery
center between stages 2 and 3; the shunt was occluded at the time of
IECF. One patient (pt. no 17) had interrupted inferior vena cava with
azygos vein continuity and Kawashima procedure was performed at stage 2.
Patients no 1 and 10 had bilateral superior vena cava that were
anastomosed to the ipsilateral pulmonary arteries at stage 2.
Stage-3 palliation included fenestrated IECF completion procedure in all
cases. A polytetrafluorethylene (PTFE) tubular conduit
(GORE-TEX, W.L. Gore and Associates Ltd., Livingston,
Scotland) with a size of 18 or 20 mm was used in all cases. A
fenestration of 4, 4.5 or 5 mm was performed with an aortic punch.
Concomitant procedures at stage III included atrial septectomy (pt.no 1
and 2), AV valve repair (pt.no 9, 10 and 15) and pulmonary artery
reconstruction (pt.no 12 and 15). All of the patients were evaluated
with cardiac catheterization before Fontan completion. The mean
pulmonary artery (Nakata) index was 209 ± 67
mm2/m2 (range: 97 – 315
mm2/m2). Mean preoperative pulmonary
artery pressure that was directly measured at catheterization was 13.8 ±
3.3 mmHg (range: 8 – 21 mmHg), whereas the postoperative MPAP measured
at the central venous line was 10 ± 2.4 mmHg (range: 4 – 14 mmHg). The
stages of Fontan circulation and concomitant procedures are summarized
in table-2.