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Published on: August 26, 2025
Predicting Outcomes in Pediatric Intraluminal Pulmonary Vein Stenosis Using a Comprehensive Standardized
Ryan Callahan1, Kimberlee Gauvreau2, Mirjam Keochakian2
1Department of Pediatrics, Children's Hospital of Philadelphia, and University of Pennsylvania Perelman School of Medicine (R.C.).
Insights
Pulmonary vein stenosis (PVS) in children can be assessed using multimodal catheterization. Angiography and intravascular ultrasound findings predict vein outcomes, aiding in risk stratification for improved patient management.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Vascular Biology
Background:
- Pediatric intraluminal pulmonary vein stenosis (PVS) affects a diverse group of young patients.
- The hemodynamic and physiological impacts of PVS on pulmonary veins, including lumen, pressure, wall structure, compliance, and shear stress, require further investigation.
Purpose of the Study:
- To assess PVS characteristics using a multimodal catheterization approach.
- To determine the association between PVS characteristics and vein outcomes in pediatric patients.
Main Methods:
- A prospective trial involving 50 pediatric patients (<13 years) undergoing cardiac catheterization for PVS.
- Utilized angiography, pulmonary vein pressure, intravascular ultrasound, balloon compliance testing, and wall shear stress calculations.
- Assessed vein status at 12 months as the primary outcome and transplant-free survival as the secondary outcome.
Main Results:
- Increased angiographic severity, greater wall thickness (intravascular ultrasound), higher postintervention gradient, and elevated wall shear stress correlated with worse vein outcomes.
- Multivariable analysis identified upstream luminal narrowing, ostial diameter <6 mm, and ≥35% upstream wall thickness as significant predictors of adverse vein outcomes.
- A 3-point Invasive Pulmonary Vein Score prediction tool was developed; 12-month transplant-free survival was 92%.
Conclusions:
- Multimodal pulmonary vein catheterization, particularly angiography and intravascular ultrasound, effectively predicts 12-month pulmonary vein outcomes in pediatric PVS.
- The findings support the use of these techniques for risk stratification and management of pediatric PVS.
Background:
Pediatric intraluminal pulmonary vein stenosis (PVS) occurs in a heterogeneous patient population but is unified by its cellular composition in affected pulmonary veins. Enhanced delineation of the hemodynamic and physiological vein consequences of PVS and its impact on vein lumen, vein pressure, wall structure, vein compliance, and wall shear stress has not been studied. The objective is to assess PVS characteristics using multimodal catheterization techniques (angiography, pulmonary vein pressure, intravascular ultrasound, balloon compliance testing, and wall shear stress calculation) and determine their association with vein outcomes.
Methods:
The study is a prospective trial applying a standardized catheterization assessment to patients under 13 years of age who are undergoing cardiac catheterization for PVS. The primary outcome is vein status at 12 months, assigned by an end point committee. The secondary outcome is transplant-free survival.
Results:
Overall, 50 patients with a median age of 2.7 years (range, 0.3-11.8) underwent assessment and included angiography of 200 veins, 171 pressure assessment, 169 intravascular ultrasound, 58 balloon compliance testing, and 141 wall shear stress calculations. The probability of a worse vein outcome increased as angiographic severity score, percent wall thickness by intravascular ultrasound, postintervention gradient, or postintervention wall shear stress increased. Multivariable analysis demonstrated veins with angiographic upstream luminal narrowing (odds ratio, 3.26 [95% CI, 1.38-7.7]; P=0.007), ostial diameter <6 mm (odds ratio, 3.93 [95% CI, 1.29-12]; P=0.016), and ≥35% upstream wall thickness by intravascular ultrasound (odds ratio, 5.05 [95% CI, 2.03-12.5]; P<0.001) are associated with a worse vein outcome. A 3-point Invasive Pulmonary Vein Score prediction tool was created. Transplant-free survival was 92% at 12 months.
Conclusions:
A multimodal pulmonary vein catheterization assessment determined angiographic and intravascular ultrasound findings to be the most predictive of 12-month pulmonary vein outcome.
Registration:
URL: https://www.clinicaltrials.gov; Unique identifier: NCT04696289.
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