Mid-term outcomes of a catheter-based right ventricular decompression strategy for pulmonary atresia with intact ventricular septum and critical pulmonary stenosis

Abstract Pulmonary atresia with intact ventricular septum (PAIVS) and critical pulmonary stenosis (cPS) span a spectrum of right ventricular hypoplasia in which the optimal initial pathway remains uncertain. We describe mid-term outcomes of an anatomy-guided percutaneous transcatheter pulmonary valvuloplasty (PTPV)-first strategy. We retrospectively reviewed 24 patients treated invasively from 2007 through 2025; three lacked measurable right ventricular end-diastolic volume (RVEDV) and were excluded, leaving an analytic cohort of 21 patients (PAIVS, n = 9; cPS, n = 12). Fifteen patients with a tripartite right ventricle and no major sinusoidal communication underwent PTPV first; six with an absent outlet component or major sinusoidal communication entered a single-ventricle pathway. All 15 PTPV-first patients achieved biventricular circulation; all three PAIVS patients in this group required subsequent, elective surgical right ventricular decompression, consistent with staged rather than failed decompression. All six single-ventricle patients completed Fontan circulation, and no PTPV-first patient required one-and-a-half ventricular repair or crossed over to Fontan. All 21 patients were alive after a median follow-up of 8 years [interquartile range, 6–11]. One patient with borderline RVEDV and tricuspid valve Z-score developed protein-losing enteropathy 11 years after Fontan completion. PTPV-first patients tended to have higher RVEDV and less negative tricuspid valve Z-scores than single-ventricle patients, reflecting selection criteria rather than validated thresholds. PTPV first permitted staged right ventricular recruitment and biventricular circulation with favorable mid-term outcomes, with surgical decompression as an effective subsequent step. Given the small, retrospective, single-center design, these findings are hypothesis-generating and warrant prospective reassessment of borderline right ventricles.