KẾT QUẢ TRUNG HẠN SAU PHẪU THUẬT SỬA CHỮA HOÀN TOÀN TẬT KHÔNG LỖ VAN ĐỘNG MẠCH PHỔI KÈM THÔNG LIÊN THẤT TẠI BỆNH VIỆN NHI ĐỒNG 1

Abstract

Background: In PA-VSD, as well as conotruncal anomalies, the major postoperative concerns after complete repair are dysfunction of the RV–PA conduit and RV dysfunction. Evaluation of mid-term outcomes is essential for follow-up and timely intervention.

Objective: To evaluate mid-term outcomes after complete repair of PA-VSD by determining the rates of anatomical pulmonary artery stenosis, conduit valve stenosis and regurgitation, RVOT aneurysm, RV dysfunction, and the need for reintervention during 1–5 years of follow-up.

Method: A descriptive case series including all pediatric patients with PA-VSD who underwent complete surgical repair with RV–PA conduit placement and VSD closure at Children’s Hospital 1 from January 2019 to April 2024.

Results: Thirty-five patients were included, of whom 97.1% had Castaneda type I or II anatomy. Most patients underwent surgery between 6 months and 2 years of age, with a median weight of 9 kg and a mean preoperative oxygen saturation of 76%. Preoperative palliative interventions were performed in 97.1% of cases, mainly ductal stenting. The RV–PA conduit was selected with a mean z-score of +2, and the median postoperative RV-to-LV pressure ratio was 0.27. During 1–5 years of follow-up, mild heart failure symptoms were observed in 11.4% of patients, 54.3% had right bundle branch block, and no deaths were recorded. Left pulmonary artery stenosis was more frequent than the right one (50% vs. 10.7%). RV–PA conduit stenosis occurred in 65.7%, with moderate-to-severe stenosis in 45.7%, predominantly involving the distal segment. Moderate-to-severe conduit valve stenosis was present in 51.4%, whereas conduit valve regurgitation was mostly mild. RVOT dilation was observed in 34.3%, mainly mild. RV enlargement occurred in 71.4%. RV systolic dysfunction was detected in 74.3% by echocardiography and 85.7% by cardiac magnetic resonance (CMR), while RV diastolic dysfunction was in 82.9% by echocardiography. Significant abnormalities could emerge as early as 1 year postoperatively. Reintervention was required in 60% of patients, mostly at 2.5 years after surgery, primarily due to RV–PA conduit stenosis, followed by PA branch stenosis.

Conclusion: Reintervention due to RV–PA conduit stenosis remains highly prevalent after complete repair of PA/VSD. Therefore, long-term follow-up with multiparametric echocardiography combined with cardiac magnetic resonance is essential for early detection of complications and timely reintervention.