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.