Daiji Takajo, Paul J. Critser, Awais Ashfaq, Russel Hirsch
Cincinnati Children’s Hospital Medical Center and University of Cincinnati. University of Texas Southwestern Medical Center.
United States
Pediatric Cardiology
Pediatr Cardiol 2026;
DOI: 10.1007/s00246-026-04266-4
Abstract
Background: Pulmonary vein stenosis (PVS) is a rare but serious condition in children, often requiring surgical or catheter-based interventions. The initial optimal treatment strategy remains unclear due to disease complexity, progression, and high rates of recurrence.
Methods: In this retrospective, single-center study, we identified children with primary or secondary PVS from a cardiac catheterization and surgical database between 2015 and 2023. Patients with single ventricle physiology were excluded. Demographics and outcomes were compared between patients who underwent catheter-based intervention only and those who underwent at least one surgical pulmonary vein repair, with or without subsequent catheter-based reintervention. Reintervention following surgical repair was assessed using Kaplan-Meier analysis.
Results: Among 56 children with biventricular physiology and PVS (33 males, 59%), 16 (29%) underwent at least one surgical repair at a median age of 9 months (IQR 4-20), while 40 (71%) were managed with catheter-based interventions alone. Surgical repair was more frequently performed in patients with bilateral or complex disease, particularly those without prematurity or with coexisting congenital heart defects requiring open-heart surgery. Over time, catheter-based approaches became increasingly preferred. Overall, 92% of surgical patients required reintervention, most within the first year. Mortality did not significantly differ between groups (p = 0.294). In the surgical group, elevated right ventricular/systemic systolic pressure ratio (HR: 1.25, p = 0.048) and the presence of scimitar syndrome (HR: 8.25, p = 0.011) were associated with increased mortality.
Conclusions: Surgical pulmonary vein repair remains an important option, particularly in cases where catheter-based intervention is not feasible due to anatomical challenges or when multiple pulmonary veins are severely affected. However, recurrent pulmonary vein re-intervention is common, regardless of whether the initial approach was surgical or catheter-based.
Category
Stenosis or Obstruction of Normal Pulmonary Venous Connections
Stenosis or Obstruction of Pulmonary Veins Following Surgical Repair of Anomalous Pulmonary Venous Connections
Catheter-mediated Interventions: Efficacy or Lack of Efficacy
Surgical Interventions for Pulmonary Venous Obstruction After the Onset of Disease
Year of Publication: 2026
Age Focus: Pediatric
Article Type: Retrospective Observational Cohort Studies (>10 patients)
Article Access: Free PDF File or Full Text Article Available Through PubMed or DOI: Yes