Results
Operative and postoperative results are listed in table 2. Mean BSA of the patients in group 1 was lower than group 2 (p=0.044), and the number of the premature patients was higher in group 1 (p=0.056). In group 1, 4 patients underwent atrial septectomy, and 2 underwent aortic valve commissurotomy because of bicuspid aortic valve, additionally. Pulmonary artery banding was performed to one of these two patients who underwent aortic valve commissurotomy due to multiple VSD. In group 2, 18 patients underwent VSD closure; 12 patients underwent arterial switch operation (ASO) with VSD closure; one underwent aorto-pulmonary window repair. Although 3 of the remaining 6 patients had a bicuspid aortic valve, the valve did not need commissurotomy, and just like 3 patients with isolated arch hypoplasia, these patients underwent isolated aortic arch repair. The mean ASCP times were 22.4 ±7.4 min. The mean CPB and aortic cross-clamping (CC) times were significantly longer in group 2 (CPB time: 56.9 ± 10.4 min in group 1 versus 108.7 ± 50.5 min in group 2, p<0.001; CC time: 12.6 ± 6.5 min in group 1 versus 54.4 ± 36 min in group 2, p<0.001).
In group 1, 10 patients underwent pulmonary artery banding despite having functional biventricle and VSD. Of these 10 patients, 4 (40%) were premature, 6 (60%) had low body weight (≤ 2.5 kg), and 3 (30%) had poor preoperative condition or required mechanical ventilation support. Only 3 patients did not have any comorbidities and VSDs closing were postponed to second stage due to anatomical location or size. They had large subaortic, apical musculer and large inlet-outlet lying VSDs, respectively.
Hospital mortality were 34.8 % and 10.8% respectively (8/23 in group 1 and 4/37 in group 2, p=0.024). Of the 8 patients in group 1, 6 (75%) were neonate, 4 (50%) were male, 4 (50%) had low body weight (<2.5 kg), 5 (62.5%) had functional single ventricle, and 5 (62.5%) had preoperative poor condition or required mechanical ventilation support. Bleeding complication was occurred in 2 patients, low cardiac output was occurred in 3 patients, and they died due to sepsis in the postoperative course. One patient had sudden cardiac arrest on the postoperative day 2 and died despite resuscitation. Extracorporeal membrane oxygenation (ECMO) support did not use in this patient because of low body weight and poor preoperative condition. The remaining 2 patients died due to Klebsiella pneumoniae sepsis and Enterococcus faecium mediastinitis after the long postoperative course, respectively. Of the 4 patients in group 2, all patients were neonate and male, 1 (25%) had low body weight (<2.5 kg), and 3 (75%) needed preoperative sepsis treatment or required mechanical ventilation support. One patient underwent reoperation due to mitral valve insufficiency after the perimembranous VSD closure, this patient and another patient died because of sepsis after long postoperative course. ECMO support was used in the remaining 2 patients due to low cardiac output. One of them weaned from ECMO but could not survive and died due to multi organ failure, the another one died on ECMO because of Klebsiella pneumoniae sepsis.
The median duration of intensive care unit (ICU) and hospital stay were 11 days (range 2-228), and 19 days (range 2-230), respectively. Although the median length of ICU stay was not statistically significant, was longer in group 1 (15 days to 10 days, p= 0.45). In addition, the median length of hospital stay was not different between two groups (19 days to 19 days, p= 0.93). Sternal closure was delayed in 35 cases (58,3%) by a mean of 4.3 ± 5.9 days (range 1 to 33), and there was no statistically significant difference between two groups (12/23 in group 1 and 23/37 in group 2, p=0.44). However, the mean duration of delayed sternal closure time was significantly higher in group 1 (6.8±8.7 versus 2.7±2.1, p=0.044). Twenty-three patients (39.6%) (10/23 versus 13/37, p=0.48) needed a long duration of mechanical ventilation (>7 days), and twenty-four patients needed prolonged ICU stay (>15 days) (12/23 versus 11/37, p=0.12). VAC therapy was performed due to mediastinitis or delayed sternal closure in 5 cases (8.3%), and was significantly higher in group 1 (4/23 versus 1/37, p=0.045). Three patients (5%) needed re-exploration for bleeding, and two patients (3.3%) experienced supraventricular dysrhythmias. Eight patients (13.3%) showed postoperative low cardiac output syndrome, and that was statistically significantly higher in group 1 (6/23 versus 2/37, p=0.022). Sudden cardiac arrest was occurred in 4 cases (6.6%), and all of them were in group 1 (p=0.009). Two patients needed postoperative ECMO support due to extracorporeal cardiopulmonary resuscitation or low cardiac output. Unfortunately, although one patient weaned from ECMO, both patients died due to sepsis. Tracheostomy was required due to the long duration of mechanical ventilation in four cases (6.6%). Sepsis was occurred in 7 cases (11.6%) (5/23 versus 2/37, p=0.055), and one of them had chylothorax. Two patients underwent left diaphragm plication operation because of left diaphragm paralysis in the early postoperative period. Peritoneal dialysis was required in 3 cases (5%) because of renal insufficiency and/or positive fluid balance. Minor neurological events occurred in 3 cases (5%). Three patients (5%) were needed antihypertensive therapy while discharged from the hospital. Outcomes and complications were presented in Table 3.
Follow-up was completed for 93,75% (45/48) survivors with a mean duration of 21.9 ±15.1 months (range 2 to 58 months). Kaplan-Meier survival for both groups is shown in Figure 2, and there was an early survival advantage in group 2 (p=0.019). Risk factors affecting survival are listed in Table 4. No mortality was observed during follow-up period.
Recoarctation was occurred in 13 cases (27%) and 11 (22.9%) of them required reintervention (Figure 3). We used glutaraldehyde treated autologous pericardium in 21 patients, bovine pericardium in 19 patients, porcine pericardium in 20 patients, and there was no statistically significant relationship between patch types and recoarctation (p=0,77). Moreover, there was no association between Z scores of the proximal aortic arch, distal aortic arch, transverse aortic arch, and recoarctation. Similarly, the incidence of re-intervention was not statistically significant between the two groups (p=0,89) (Figure 4). Among 13 patients, 7 patients underwent balloon dilation angioplasty, 4 patients required reoperation as patch aortoplasty after unsuccessful balloon dilatation, and the last 2 patients were closely followed up by medical treatment. The mean of arch reintervention time was 8.7 ± 7.3 months (range 2 to 27 months), and the angioplasty evaluation of the mean gradient 38.5 ± 15.5 mm Hg dropped to the mean of 6.1 ± 4.5 mmHg gradient by balloon angioplasty. One case required stent implantation (9x17 mm stent; Visi-pro, Medtronic, USA) to the descending aorta after surgical reintervention. There were no cases of bronchial compression or aneurysm of the aortic arch. Patients required reinterventions were presented in table 5.
Of the remaining 15 patients (65.2%) in group 1, two were lost to follow-up. Two patients underwent pulmonary debanding with VSD closure. Two underwent transcatheter pulmonary debanding, and remaining small muscular VSDs were following-up. One patient underwent Yasui procedure, one underwent Glenn with Damus-Kaye-Stansel anastomosis, one underwent pulmonary debanding with AVSD repair. All patients were survived after the second stage operation, and were in good clinical condition. The remaining six patients were under follow-up for the second stage operation.