신생아 및 조기 영아에서의 심실중격결손을 동반한 폐동맥 폐쇄에 대한 외과적 전략: 단계별 교정술과 일차 완전 교정술에 대한 비교

Abstract

Background: Initial surgical options for pulmonary atresia with ventricular septal defect (PA/VSD) in neonates and young infants are either palliative shunt operation or early total correction. Staged repair strategy may be associated with a higher risk of inter-stage mortality, and primary repair strategy may lead to frequent post-repair reinterventions. Methods: From 2004 to 2017, 65 patients with PA/VSD who underwent surgical intervention before the age of 90 days were identified and enrolled in this retrospective study. The cohort was divided into two groups; group R who underwent primary repair (n=15), and group P who underwent Blalock-Taussig shunt or right ventricle to pulmonary artery (RV-PA) conduit (n=50). Risk of multiple reoperations among the survivors was analyzed using Prentice-Williams-Peterson (PWP) model, and Cox proportional hazards model was fitted to determine the risk factors for decreased time to composite adverse outcome (death, reoperations) after birth. Results: Median follow-up was 42.9 (IQR 19.4-98.7) months. Two groups did not differ in age at initial operation (Group R: 36.5 ± 27.9 days, Group P: 28.4 ± 15.6 p=0.298), BSA (Group R: 0.2 ± 0.0, Group P: 0.2±0.0, p=0.105), but differed in pulmonary artery index (Group R: 164.5±51.9 mm2/m2, Group P: 124.6±50.9 mm2/m2, p=0.010). During the follow-up duration, there were nine surgical mortalities (Group P=7, Group R=2), 24 first reoperations after repair (Group P=16, Group R=8), and 11 second reoperations (Group P=3, Group R=8). Five-year survival was comparable between the two groups (group R=86.7%, group P=83.6%, p=0.754), but staged repair showed a decreased risk of multiple reoperations compared to primary repair (HR 2.73, 95% CI:0.2751-0.7204, p=0.0086). Cox model showed primary repair as the only risk factor for decreased time to death/first reoperations (HR 2.53, 95% CI:1.002-5.453; P=0.049) and death/second reoperations (HR 2.90, 95% CI:1.09-7.75, p=0.026) after birth. Conclusions: Staged repair strategy, as compared to initial total repair, was associated with higher inter-stage mortality with less frequent reinterventions after repair, which may be attributable to the use of larger conduits upon repair. Lowering the inter-stage mortality in the staged repair may allow for better surgical outcome in the future.