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

Background: We aimed to explore the relation between temporal right heart growth and long-term outcomes in patients with pulmonary atresia with an intact ventricular septum (PA/IVS) who underwent balloon pulmonary valvuloplasty (BPV).

Methods: We performed echocardiography to measure pulmonary valve diameter, right atrial end-systolic area (RAA), and right ventricular end-diastolic area (RVA) before BPV 1 and 5 years after BPV. Primary and secondary end points were to explore temporal changes in right heart structures and to determine echocardiographic parameters related to late adverse events (LAEs).

Results: In 31 patients, pulmonary valve diameter significantly increased after BPV, whereas tricuspid valve diameter remained unchanged throughout the 10.0-year follow-up (range, 5.8-14.0 years). After BPV, RAA temporally decreased, whereas RVA significantly increased. There were LAEs in 6 patients (19%), arrhythmias in 2, heart failure in 1, reintervention of the right ventricular outflow tract in 1, and reintervention for residual cyanosis in 2. The rate of freedom from LAEs at 5 and 10 years was 92% and 82%, respectively. Right atrial end-systolic area temporally decreased in patients without LAEs (P < .01); however, RAA remained unchanged throughout the period in patients with LAEs (P = .16). Moderate or severe pulmonary regurgitation (hazard ratio = 23.0; 95% confidence interval, 1.3-385; P = .03) and the ratio of RVA to RAA at 1 year after BPV (hazard ratio = 6.3 × 10; 95% confidence interval, 2.1 × 10 to 0.19; P = .03) were independent risk factors for LAEs.

Conclusions: Disproportional right heart growth was observed in patients with pulmonary atresia with an intact ventricular septum after BPV. Pulmonary regurgitation and increased RAA are crucial for identifying the burden of LAEs among them.