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Updated: Nov 10, 2025
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Published on: August 26, 2025
Outcomes in Establishing Individual Vessel Patency for Pediatric Pulmonary Vein Stenosis
Ryan Callahan1, Kimberlee Gauvreau1, Audrey C Marshall1
1Department of Cardiology, Boston Children's Hospital and Harvard Medical School, Boston, MA 02115, USA.
Insights
Predictors of minimal disease in pediatric pulmonary vein stenosis (PVS) include lack of distal disease, specific vein locations, and patient age. Higher drug doses correlated with fewer reinterventions for PVS patients.
Area of Science:
- Cardiology
- Pediatric Pulmonology
- Clinical Pharmacology
Background:
- Intraluminal pulmonary vein stenosis (PVS) is a complex condition in pediatric patients.
- Effective treatment strategies for multi-vessel PVS are crucial for improving patient outcomes.
- Understanding predictors of individual vein outcomes is essential for personalized treatment approaches.
Purpose of the Study:
- To identify patient and pulmonary vein characteristics predictive of outcomes in pediatric intraluminal pulmonary vein stenosis (PVS).
- To evaluate the impact of baseline disease characteristics and treatment intensity on PVS progression and reintervention rates.
Main Methods:
- Retrospective sub-analysis of individual pulmonary veins from a clinical trial (NCT00891527) involving pediatric PVS patients treated with imatinib mesylate +/- bevacizumab.
- Analysis of 182 pulmonary veins from 46 patients over a 72-week follow-up period.
- Multivariable analysis to determine predictors of minimal disease and inverse regression analysis for reintervention rates.
Main Results:
- Absence of distal disease at baseline, non-left upper vein location, and patient age ≥ 1 year predicted higher odds of minimal disease at 72 weeks.
- Higher percentage of eligible drug doses received was associated with significantly fewer reinterventions (IRR 0.76, p < 0.001).
- Baseline characteristics and treatment intensity influenced individual vein outcomes in pediatric PVS.
Conclusions:
- Successful multi-modal treatment for aggressive pediatric PVS is influenced by vein location, disease severity, and drug dose intensity.
- Identifying predictive factors can guide therapeutic decisions and improve management of pediatric pulmonary vein stenosis.
- Further research into optimizing adjunct therapies for PVS is warranted.
Abstract:
The purpose of this study was to determine what patient and pulmonary vein characteristics at the diagnosis of intraluminal pulmonary vein stenosis (PVS) are predictive of individual vein outcomes. A retrospective, single-center, cohort sub-analysis of individual pulmonary veins of patients enrolled in the clinical trial NCT00891527 using imatinib mesylate +/- bevacizumab as adjunct therapy for the treatment of multi-vessel pediatric PVS between March 2009 and December 2014 was performed. The 72-week outcomes of the individual veins are reported. Among the 48 enrolled patients, 46 patients and 182 pulmonary veins were included in the study. Multivariable analysis demonstrated that patients with veins without distal disease at baseline (odds ratio, OR 3.69, 95% confidence interval, CI [1.52, 8.94], p = 0.004), location other than left upper vein (OR 2.58, 95% CI [1.07, 6.19], p = 0.034), or veins in patients ≥ 1 y/o (OR 5.59, 95% CI [1.81, 17.3], p = 0.003) were at higher odds of having minimal disease at the end of the study. Veins in patients who received a higher percentage of eligible drug doses required fewer reinterventions (IRR 0.76, 95% CI [0.68, 0.85], p < 0.001). The success of a multi-modal treatment approach to aggressive PVS depends on the vein location, disease severity, and drug dose intensity.
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