Outcomes in Establishing Individual Vessel Patency for Pediatric Pulmonary Vein Stenosis.

Ryan Callahan,Christina M Ireland,Christopher W Baird,Kimberlee Gauvreau,Juliana T Mendonca,Laureen M Sena,Audrey C Marshall,Kerry Mcenaney,Elsa C Bjornlund,Kathy J Jenkins

doi:10.3390/children8030210

Ryan Callahan, Christina M Ireland + Show 8 more

Open Access

https://doi.org/10.3390/children8030210

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Journal: Children	Publication Date: Mar 10, 2021
Citations: 6	License type: CC BY 4.0

Affiliation: Boston Children's Hospital, Harvard University

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.

Highlights

Pediatric intraluminal pulmonary vein stenosis (PVS) is caused by the hyperplasia of myofibroblast-like cells in a myxocollagenous matrix which compromises flow and leads to pulmonary hypertension, right heart failure, and death [1,2,3]
As patient survival is dependent on the preservation of as much healthy pulmonary vein bed as possible, identifying individual pulmonary vein characteristics associated with vein outcomes may assist in improving patient survival
Most patients were less than one year of age (36/46; 78%), had four vessel involvement at baseline (25/46; 54%), and had PVS associated with congenital heart disease (37/46; 80%, 16 of which had anomalous pulmonary venous connections)

Summary

Introduction

Pediatric intraluminal pulmonary vein stenosis (PVS) is caused by the hyperplasia of myofibroblast-like cells in a myxocollagenous matrix which compromises flow and leads to pulmonary hypertension, right heart failure, and death [1,2,3]. Factors associated with patient survival include age, overall disease burden, the severity of pulmonary hypertension, and right ventricular function [4,5,6,7,8,9]. Aggressive subtypes involve multiple veins and have a high incidence of restenosis, irrespective of intervention [10,11,12]. In these cases, a multi-modal approach using transcatheter and surgical techniques combined with adjunct anti-proliferative therapy is implemented in an effort to improve survival [13]. We hypothesized that pulmonary vein location and pulmonary vein disease severity at baseline would predict vein outcome

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