Late Adverse Events in Patients With Pulmonary Atresia With Intact Ventricular Septum After Valvuloplasty

Abstract Background We aimed to explore the relationship between temporal right heart growth and long―term outcomes in patients with pulmonary atresia with intact ventricular septum (PA/IVS) who underwent balloon pulmonary valvulopalsty (BPV). Methods Echocardiography was performed to measure the pulmonary valve diameter (PVD), the right atrial end―systolic area (RAA), and the right ventricular end―diastolic area (RVA) before BPV, 1 and 5 years after BPV. The primary and secondary endpoints were to explore temporal changes in the right heart structures and to determine echocardiographic parameters related to late adverse events (LAEs). Results In 31 patients, PVD significantly increased after BPV, whereas TVD remained unchanged throughout the follow―up period of 9.1 (5.7―12.0) years. After BPV, RAA temporally decreased, whereas RVA significantly increased. There were six LAEs (19%); arrhythmias in two, heart failure in one, reintervention of the right ventricular outflow tract in one, and reintervention for residual cyanosis in two. The rate of freedom from LAEs at 5 and 10 years were 92% and 82%, respectively. RAA temporally decreased in patients without LAEs (P Conclusions Disproportional right heart growth was observed in patients with PA/IVS after BPV. PR and increased RAA are crucial in identifying the burden of LAEs among them.