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.