Improving outcomes of patients with heterotaxy and functional single ventricle: a 10-year follow-up of 70 cases in a single institution / 中华外科杂志

ABSTRACT

Objective@#To review current-era palliation outcomes of patients with heterotaxy and functional single ventricle in a single institution.@*Methods@#The clinical data of 70 consecutive patients with heterotaxy undergoing multistage single ventricle palliation in Guangzhou Women and Children′s Medical Center from January 2008 to December 2017 were retrospectively reviewed, and the prognosis factors for mortality were analyzed. There were 53 male and 17 female patients.The median age was 13.3 months (range: 6 days to 150 months). Single ventricle multistage palliation included 1st stage palliation surgery, 2nd bidirectional Glenn shunt, and 3rd stage modified Fontan.The Kaplan-Meier method was used to estimate the probability of survival. Multivariate analysis was performed by Cox regression model.@*Results@#Sixty-two patients had right atrial isomerism while eight had left atrial isomerism. Eighteen patients (25.7%) required the first stage palliation. Fifty-five patients received the Glenn shunt, and the Fontan procedure was completed in 27 patients. Overall, mortality occurred in 17 patients (24.3%) including 9 after the first stage palliation, 6 after the Glenn shunt, and 2 following the Fontan procedure. Survival estimates for the entire cohort following surgery were 81% (95%CI: 72% to 90%), 74%(95%CI: 64% to 85%), and 74% (95%CI: 64% to 85%) at 1 year, 5 years and 10 years, respectively. Survival estimates following the first stage palliation were 56% (95%CI: 33% to 79%) and 49% (95%CI: 26% to 73%) at 1 year and 5 years, respectively. Multivariate Cox regression analysis revealed that total anomalous pulmonary venous connection (TAPVC) (HR=6.16, 95%CI: 1.65 to 22.95, P=0.007), atrioventricular valve regurgitation more than moderate (HR=3.81, 95%CI: 1.32 to 10.94, P=0.013) and required the first palliation surgery (HR=4.58, 95%CI: 1.34 to 15.72, P=0.016) were prognosis factors for overall mortality.@*Conclusions@#The management of heterotaxy patients with functional single ventricle remains challenging, and the outcomes are continously improving in china. TAPVC, atrioventricular valve regurgitation more than moderate and required the first palliation surgery still are prognosis factors for overall mortality in heterotaxy syndrome.