Ashish Saini, Hui Huang, Andrew Jergel, Rosemary Gray, Dennis W. Kim, James Kuo
Children’s Healthcare of Atlanta and Emory University School of Medicine.
United States
Pediatric Cardiology
Pediatr Cardiol 2026;
DOI: 10.1007/s00246-026-04350-9
Abstract
Pediatric pulmonary vein stenosis (PVS) is a rare, progressive condition associated with significant morbidity and mortality. While pulmonary venous decompressing collaterals (PVDC) have been described, their clinical relevance and impact on long-term outcomes remain unstudied. We aimed to characterize these collaterals and determine their association with outcomes and mortality in pediatric PVS. This was a single-center, retrospective cohort study of 64 pediatric patients (≤ 18 years) treated for PVS between 2017 and 2022. Patients with single-ventricle heart disease were excluded. PVDC were defined as contrast flow to a neighboring lobar or interlobar segment due to medial occlusion identified on serial angiograms. The primary outcome was PVS-related mortality analyzed using multivariable Cox proportional hazards models, adjusting for age and gestational age. PVDC were present in 39/64 (61%) patients. The presence of collaterals was significantly associated with lower all-cause (15% vs. 40%, p = 0.027) and PVS-related mortality (13% vs. 40%, p = 0.012). This benefit was most pronounced in the bilateral disease subgroup (45% of the cohort). In this group, patients with collaterals had significantly lower PVS-related mortality (15% vs. 78%, p = 0.002) and lower indexed pulmonary vascular resistance (iPVR 3.2 vs. 7.6 Wood unit x m2, p = 0.006). In the adjusted Cox model, collateral presence in bilateral PVS was strongly associated with improved survival (HR 0.09, 95% CI: 0.02-0.46, p = 0.003). In conclusion, pulmonary venous decompressing collaterals are a favorable adaptive mechanism in pediatric PVS. Their presence is associated with improved survival, particularly in high-risk patients with bilateral disease. These findings emphasize the importance of integrating collateral status into prognostic assessment and therapeutic decision-making.
Category
Stenosis or Obstruction of Normal Pulmonary Venous Connections
Stenosis or Obstruction of Pulmonary Veins Following Surgical Repair of Anomalous Pulmonary Venous Connections
Pulmonary Hypertension Associated with Stenosis or Atresia of Pulmonary Veins
Patient Factors Influencing the Onset, Severity or Outcome of Disease
Length of Life Associated with Pulmonary Venous Obstruction
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: No