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Korean J Pediatr. 2018 Jul;61(7):210-216. English. Original Article. https://doi.org/10.3345/kjp.2018.61.7.210
Jo TK , Suh HR , Choi BG , Kwon JE , Jung H , Lee YO , Cho JY , Kim YH .
Department of Pediatrics, School of Medicine, Kyungpook National University, Daegu, Korea. kimyhmd@knu.ac.kr
Thoracic and Cardiovascular Surgery, School of Medicine, Kyungpook National University, Daegu, Korea.
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.

Copyright © 2019. Korean Association of Medical Journal Editors.