Treatment outcomes of pulmonary atresia with intact ventricular septum in newborns
Objective To summarize the treatment experiences in pulmonary atresia with intact ventricular septum(PA/IVS)in newborns.Methods Clinical data of 16 newborns with PA/IVS treated between January 2017 and January 2023 were retrospectively analyzed.There were 9 males and 7 females,with the age ranging from 4 hours to 25 days,body weight on admission ranging from 1.6kg to 4.0kg and a mean weight of(3.22±0.65)kg.All participants were followed up fora median of 33(12,55)months,ranging from 3 months to 6 years.Treatment approaches included atrial septum enlargement surgery under an extracorporeal circulation,BT shunt,hybrid therapy,and transthoracic pulmonary valve incision without cardiopulmonary bypass.One or more surgeries was performed in each neonate.Results One of the 16 children died in the hospital,and there were no deaths outside the hospital.Six cases were re-treated,including 5 treated with balloon valvuloplasty and 1 with bidirectional Glenn procedure.Among the remaining 9 newborns,1 has been lived 5 years post-operatively without the need of re-interventions,and the transcutaneous oxygen saturation was 95％and above and the interatrial septum returned a left-to-right shunt.The remaining newborns have been followed up,and they may have re-interventions.After one month of the first radical treatment,there was a large possibility to perform a biventricular radical surgery later for a larger diameter of the pulmonary artery root.Conclusion Sequential therapy and observation for PA/IVS are necessary.An individualized initial intervention based on the development of the right ventricle plus a regular active follow-up for a potential re-intervention ensure a high quality of life.
pulmonary atresia with intact ventricular septumnewbornshybrid therapysurgical treatmentfollow-up
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