Objective To comprehensively analyze the clinical outcomes of total cavopulmonary connection (TCPC) in the treatment of functional single ventricle combined with heterotaxy syndrome (HS). MethodsA retrospective analysis was conducted on the patients with functional single ventricle and HS who underwent TCPC (a HS group) in Guangdong Provincial People's Hospital between 2004 and 2021. The analysis focused on postoperative complications, long-term survival rates, and identifying factors associated with patient survival. Early and late postoperative outcomes were compared with matched non-HS patients (a non-HS group). Results Before propensity score matching, 55 patients were collected in the HS group, including 42 males and 13 females, with a median age of 6.0 (4.2, 11.8) years and a median weight of 17.0 (14.2, 28.8) kg. Among the patients, there were 53 patients of right atrial isomerism and 2 patients of left atrial isomerism. Eight patients underwent TCPC in one stage. TCPC procedures included extracardiac conduit (n=39), intracardiac-extracardiac conduit (n=14), and direct cavopulmonary connection (n=2). Postoperative complications included infections in 27 patients, liver function damage in 19 patients, and acute kidney injury in 11 patients. There were 5 early deaths. The median follow-up time was 94.7 (64.3, 129.8) months. The 1-year, 5-year, and 10-year survival rates were 87.2%, 85.3%, and 74.3%, respectively. After propensity score matching, there were 45 patients in the HS group and 81 patients in the non-HS group. Compared to the non-HS group, those with HS had longer surgical and mechanical ventilation time, higher infection rates (P<0.05), and a 12.9% lower 10-year survival rate. Multivariate Cox regression analysis identified asplenia was a risk factor for mortality (HR=8.98, 95%CI 1.86-43.34, P=0.006). ConclusionCompared to non-HS patients, patients with HS have lower survival rates after TCPC, and asplenia is an independent risk factor for the survival of these patients.
Objective To analyze the aortic development in patients with mild coarctation of the aorta (CoA) and ventricular septal defect (VSD) after isolated VSD repair and to explore the risk factors affecting postoperative aortic development. Methods A retrospective analysis was conducted on the clinical data of 4231 patients who underwent VSD repair at Guangdong Provincial People’s Hospital from January 2018 to August 2023. Patients with mild CoA were selected as the study subjects. Based on whether CoA progressed postoperatively, patients were divided into a progression group and a non-progression group. Univariate and multivariate analyses were performed, and a logistic regression model was established to analyze the factors affecting postoperative aortic development. Results A total of 231 patients were included, with 142 males and 89 females, and a median age of 223 (105, 635) days. Among the 231 patients, 30 showed varying degrees of mild CoA progression during postoperative follow-up, with an incidence rate of 13.0%. Multivariate logistic regression analysis revealed that higher preoperative pulmonary artery pressure [OR=2.053, 95%CI (1.095, 3.850), P=0.025] and larger VSD [OR=20.200, 95%CI (1.614, 254.440), P=0.020] were risk factors for postoperative CoA progression. Conclusion Most patients with mild CoA and VSD exhibited varying degrees of catch-up growth in the aorta postoperatively. Higher preoperative pulmonary artery pressure and larger VSD size are influencing factors for postoperative CoA progression, necessitating more cautious surgical strategies and closer follow-up for this subset of patients.