ĐÁNH GIÁ KẾT QUẢ SAU PHẪU THUẬT THÂN CHUNG ĐỘNG MẠCH TẠI BỆNH VIỆN NHI ĐỒNG 1

Abstract

Background: Truncus arteriosus (TA) is a rare congenital heart defect but has a guarded prognosis. Early complete surgical repair significantly improves survival; however, residual problems and late complications, especially related to the right ventricle–pulmonary artery (RV–PA) conduit and ventricular function, remain major challenges. In Vietnam, data on mid- and long-term outcomes after TA repair are still limited. This study was conducted to supplement domestic clinical evidence.

Objective: To evaluate the outcomes at ≥ 5 years after surgery in patients with Truncus arteriosus (TA) at Children’s Hospital 1 through determining the mortality rate, identifying factors associated with mortality, assessing the status of reintervention after surgery, and analyzing clinical characteristics, as well as cardiac morphology and function.

Method: A descriptive case-series study of 30 children diagnosed with TA who underwent complete repair at Children's Hospital 1 from January 2015 to September 2020. Patients were followed for more than 5 years after surgery. Clinical features, complications, reinterventions, cardiac morphology, and function were mainly assessed by echocardiography.

Results: Thirty pediatric patients were included in the study, with 40% males and 60% females. Only 3.3% of cases had a prenatal diagnosis, and 10% had DiGeorge syndrome. The median age at surgery was 81.5 (68–136) days, with a median body weight of 4.05 (3.5–5) kg. The overall mortality rate was relatively high at 32.1%. Early mortality (within the first 30 days after surgery) was 16.7%, and late mortality (after 30 days postoperatively) was 15.4%. A body weight < 4 kg at the time of surgery and severe preoperative heart failure were factors associated with mortality. After surgery ≥ 5 years, most children were asymptomatic (84.2% NYHA class I) or had only mild heart failure (15.8% NYHA class II). However, reintervention was required at a median of approximately 4 years after the initial surgery, with a high rate of up to 73.6%. Reinterventions mainly included cardiac catheterization with balloon dilation of the right ventricle–pulmonary artery (RV–PA) conduit (57.9%) or surgical replacement with a larger conduit (47.4%). Prominent cardiac morphological abnormalities included right ventricular hypertrophy (82.4%), RV–PA conduit stenosis (76.5%), and aortic dilation (70.6%). The rate of right ventricular dysfunction was relatively high (11.8%), whereas left ventricular function was generally preserved.

Conclusions: Complete repair of truncus arteriosus provides favorable mid- and long-term outcomes. Close surveillance of the right ventricle–to–pulmonary artery (RV–PA) conduit and right ventricular function is crucial to optimizing long-term outcomes for these patients.