Aspiration Is Associated with Poor Treatment Response in Pediatric Pulmonary Vein Stenosis.

Maria Niccum,Kimberlee Gauvreau,Ryan Callahan,Kathy J Jenkins

doi:10.3390/children8090783

Maria Niccum, Kimberlee Gauvreau + Show 2 more

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https://doi.org/10.3390/children8090783

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Journal: Children	Publication Date: Sep 7, 2021
Citations: 4	License type: CC BY 4.0

Affiliation: Boston Children's Hospital, Harvard University

Abstract

Intraluminal pulmonary vein stenosis is a disease with significant morbidity and mortality, though recent progress has been made using multimodal therapy with antiproliferative agents. The aim of this study was to evaluate the association between aspiration and poor treatment response in patients with intraluminal pulmonary vein stenosis. A retrospective, single-center cohort analysis was performed of patients treated with a combination of imatinib mesylate and multimodal anatomic relief between March 2009 and November 2019. Analysis focused on 2-ventricle patients due to small numbers and clinical heterogeneity of single ventricle patients. Among the 84 patients included, 15 had single ventricle physiology and 69 had 2-ventricle physiology. Among the 2-ventricle group, multivariable analysis revealed that patients with clinical aspiration had nearly five times higher odds of poor treatment response than patients without aspiration (OR 4.85, 95% CI [1.37, 17.2], p = 0.014). Furthermore, male patients had higher odds of poor treatment response than their female counterparts (OR 3.67, 95% CI [1.04, 12.9], p = 0.043). Aspiration is a novel, potentially modifiable risk factor for poor treatment response in pediatric multi-vessel intraluminal pulmonary vein stenosis in patients with 2-ventricle physiology.

Highlights

Intraluminal pulmonary vein stenosis (PVS) is a rare disease characterized by recurrent pulmonary vein obstruction due to overactivity of myofibroblast-like cells within the pulmonary vein wall [1,2]
While patients received treatment for aspiration at the discretion of treating providers, our findings suggest that clinically significant aspiration is a potentially important modifiable clinical risk factor associated with PVS outcome
In summary, two novel risk factors for poor response to multimodal PVS treatment including imatinib mesylate as an antiproliferative agent were identified for patients with 2-ventricle physiology: clinical aspiration and male sex

Summary

Introduction

Intraluminal pulmonary vein stenosis (PVS) is a rare disease characterized by recurrent pulmonary vein obstruction due to overactivity of myofibroblast-like cells within the pulmonary vein wall [1,2]. While some patients experience disease stabilization over time (evidenced by less frequent need for pulmonary vein re-interventions), a substantial proportion of patients continue to require frequent re-interventions despite maximal therapy. Several studies have evaluated risk factors for poor outcomes in PVS and have shown that patients with bilateral pulmonary vein involvement [13] and those who are smaller and younger at diagnosis have a higher risk of mortality [3]. Differences in risk for various types of lung disease have not been characterized. Since some types of lung disease are treatable, a better understanding of the role of lung disease may yield an opportune target for interventions aiming to improve PVS outcomes. The relationship between aspiration associated lung disease and PVS outcomes has not been studied

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