Surgical outcomes
Follow-up was completed for all patients (median follow-up, 29
[11-36] months). Postoperative PVO occurred in seven of 18 patients
(39%). Nine patients had preoperative PVO that necessitated
intervention for TAPVC in the neonatal period confirmed by preoperative
CT of the chest and transthoracic echocardiography. There were 14
survivors: six patients underwent the Fontan procedure, six patients
were awaiting the Fontan procedure, and two patients were awaiting the
Glenn procedure (Table 1 ). The overall survival rates at 6
months, 1 year, and 2 years were 89%, 83%, and 75%, respectively
(Fig. 2 ). There were three hospital deaths and one
post-discharge death: Patients who died in hospital were a 134-day-old
boy, a 122-day-old girl and a 93-day-old girl with supracardiac type
TAPVC and pulmonary atresia, while the patient who died after discharge
was a 91-day-old girl with supracardiac type TAPVC and mild pulmonary
stenosis. In two of three patients who died in hospital, a stent was
inserted into the obstructive vertical vein on day 0 and 28
respectively. Bilateral PAB with patent flow of the ductus artery was
performed on day 8 and 34 to regulate the PA flow respectively, and
TAPVC repair using the sutureless technique in one patient, conventional
procedure in the other patient and modified BTS were performed on day
134, 122 respectively. They died of heart failure 48 days and 80 days
after the last operation owing to a high-flow status. In the other
patient of the three, a stent was inserted into the obstructive vertical
vein on day 0, PAB was performed on day 17, and TAPVC repair using the
sutureless technique and the Glenn procedure were performed on day 91.
She died of uncontrollable chylothorax 206 days after the last
operation.
The patient who died after discharge did not have preoperative PVO and
stent insertion was not necessary. She was performed TAPVC repair using
the conventional procedure, Glenn and implantation of pacemaker because
of congenital bradycardia on day 93. While she had postoperative branch
PVOs in the apex side, postoperative course was good and she was
discharged on day 110. Fontan procedure was performed on day 478 and she
was discharged on 239 postoperative days. She died of refractory heart
failure on 295 days after Fontan procedure. Of the four dead patients,
the two patients of sutureless technique had no PVO, but the other two
of conventional procedure had PVOs.