Outcome of neonatal palliative procedure for pulmonary atresia with ventricular septal defect or tetralogy of Fallot with severe pulmonary stenosis: experience in a single tertiary center

Abstract

Purpose The present study aimed to evaluate progression and prognosis according to the palliation method used in neonates and early infants aged 3 months or younger who were diagnosed with pulmonary atresia with ventricular septal defect (PA VSD) or tetralogy of Fallot (TOF) with severe pulmonary stenosis (PS) in a single tertiary hospital over a period of 12 years.Methods Twenty with PA VSD and 9 with TOF and severe PS needed initial palliation. Reintervention after initial palliation, complete repair, and progress were reviewed retrospectively.Results Among 29 patients, 14 patients underwent right ventricle to pulmonary artery (RV-PA) connection, 11 palliative BT shunt, 2 central shunt, and 2 ductal stent insertion. Median age at the initial palliation was 13 days (1–98 days). Additional procedure for pulmonary blood flow was required in 5 patients; 4 additional BT shunt operations and 1 RV-PA connection. There were 2 early deaths among patients with RV-PA connection, one from RV failure and the other from severe infection. Finally, 25 patients (86%) had a complete repair. Median age of total correction was 12 months (range, 2–31 months). At last follow-up, 2 patients had required reintervention after total correction; 1 conduit replacement and 1 right ventricular outflow tract (RVOT) patch enlargements.Conclusion For initial palliation of patients with PA VSD or TOF with severe PS, not only shunt operation but also RV-PA connection approach can provide an acceptable outcome. To select the most proper surgical strategy, we recommend thorough evaluation of cardiac anomalies such as RVOT and PA morphologies and consideration of the patient’s condition.

Predictors of death after receiving a modified Blalock-Taussig shunt in cyanotic heart children: A competing risk analysis

“…The proportion of children receiving Glenn/biventricular repair increased around 5 months and peaked at 6.5 years (42.3%). The optimal age for the Glenn procedure is around 6–12 months (5–10 kg) in our institute which is consistent with other studies [ 14 , 15 ] whereas performing biventricular repair depends on the cardiac diagnosis [ 16 – 18 ]. However, some children had a very long delay before definite repair in our institute which could arise from many reasons.…”

Long-term outcomes of pulmonary atresia with ventricular septal defect by different initial rehabilitative surgical age

“…In our study, the estimated complete repair rate in the younger group (median age: 1.90 months) was 64% ± 8% after 3 years and 69% ± 8% after 5 years, which was significantly higher than the 28% ± 6% after 3 years and 33% ± 6% after 5 years in the elder group (median age: 33.37 months). In general, our estimated complete repair rates were somewhat lower than those in the previous studies ( 1 , 11 , 12 ). We attributed it to the criteria differences in patient enrollment.…”

Assessment of the pulmonary arterial growth and results of two-stage repair in infants with severe form of tetralogy of Fallot

“…Однако наиболее значимыми факторами риска для развития неблагоприятных событий, как при одномоментном выполнении анатомической коррекции, так и для формирования модифицированного шунта Блэлок-Тауссиг (мБТШ) являются низкий вес и выраженная гипоплазия легочного русла [1,3,8,25]. В многочисленных исследованиях, у пациентов с синдромом гипоплазии левых отделов сердца, с атрезией легочной артерии и дефектом межжелудочковой перегородки (тип A, B по Tchervenkov) выявлено, что реконструкция выходного отдела правого желудочка приводит к более эффективной реабилитации легочного русла в сравнении с формированием шунта Блэлок-Тауссиг [9][10][11][12][13][14][15][16]. Таким образом, можно предположить, что устранение выраженной обструкции выходного отдела правого желудочка при тетраде Фалло может привести к более эффективному росту центрального легочного русла в сравнении с мБТШ.…”