Experience of patent ductus arteriosus stenting in patients with a diagnosis of pulmonary artery atresia with an intact ventricular septum

Pulmonary atresia with intact ventricle septum (PA IVS) is a relatively rare congenital heart defect. This defect is characterized by the absence of flow from the right ventricle (RV) to the pulmonary artery with varying degrees of hypoplasia of the tricuspid valve (TV) and the RV cavity and has duct-dependent pulmonary circulation. Therefore, immediately after birth, the patent arterial duct (PDA) remains the only source of pulmonary blood flow. Additionally, this defect may be associated with concomitant coronary anomalies, such as ventriculocoronary fistulas, coronary artery stenosis or atresia. Aim - to evaluate the dynamics of the growth of the right heart in the remote period based on the experience of VAP stenting as a method of enriching the pulmonary blood flow in patients with ALA and MI with varying degrees of hypoplasia. Materials and methods. This retrospective single-center study included 19 consecutive patients diagnosed with PA IVS, who underwent PDA stenting at the UCC from 2015 to February 2024. Results. Perforation and balloon valvuloplasty of the pulmonary valve were performed in 9 (47.36%) patients on average for 8.6±3.52 days. Simultaneous PDA stenting with perforation and balloon valvuloplasty of the pulmonary valve was performed in 5 (26.3%) and with balloon atrial septostomy without opening antegrade flow into the pulmonary artery in 4 (21.05%) patients. Only PDA stenting was performed in 1 (5.26%) patient, who had extremely severe TV and RV hypoplasia. Conclusions. Patent ductus arteriosus stenting allows to gain time to restore the compliance of the right heart chambers and promotes the growth and development of the pulmonary blood flow. The research was carried out in accordance with the principles of the Declaration of Helsinki. Informed consent of the child and child's parents was obtained for the research. The authors declare no conflict of interest.