Outcomes in Establishing Individual Vessel Patency for Pediatric Pulmonary Vein Stenosis

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...

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Bibliographic Details
Published in:	Children (Basel) Vol. 8; no. 3; p. 210
Main Authors:	Callahan, Ryan, Gauvreau, Kimberlee, Marshall, Audrey C, Sena, Laureen M, Baird, Christopher W, Ireland, Christina M, McEnaney, Kerry, Bjornlund, Elsa C, Mendonca, Juliana T, Jenkins, Kathy J
Format:	Journal Article
Language:	English
Published:	Switzerland MDPI AG 10-03-2021 MDPI
Subjects:	Cardiovascular disease Childrens health Clinical trials Congenital diseases congenital heart disease Drug therapy Enrollments FDA approval Inhibitor drugs Lung diseases Lung transplants Medical imaging Monoclonal antibodies outcome Patients Pediatrics Pulmonary hypertension Surgery treatment Variables United States > US congenital heart disease treatment drug therapy outcome
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Summary:	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], = 0.004), location other than left upper vein (OR 2.58, 95% CI [1.07, 6.19], = 0.034), or veins in patients ≥ 1 y/o (OR 5.59, 95% CI [1.81, 17.3], = 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], < 0.001). The success of a multi-modal treatment approach to aggressive PVS depends on the vein location, disease severity, and drug dose intensity.
Bibliography:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23 Current address: Department of Cardiology, The Hospital for Sick Children, Toronto, ON M5G 1X8, Canada.
ISSN:	2227-9067 2227-9067
DOI:	10.3390/children8030210