Abstract
Pulmonary vein stenosis (PVS) is an extremely challenging clinical problem in congenital heart disease. It has traditionally required multimodal therapy given its complex underlying pathophysiology. As with other modalities, surgical therapy has undergone tremendous evolution since the 1950s. These evolving strategies have been based upon an improved understanding of the substrates that cause PVS and recurrent vein obstruction. More recent anatomic-based surgical strategies have focused on the pulmonary vein course, and how adjacent mediastinal structures can create a fulcrum effect on the pulmonary veins as they pass from the lung parenchyma to the left atrium. The consequent angulation of pulmonary veins creates altered wall shear stress and likely serves as a nidus for recurrent PVS. Encouraging early results suggest that eliminating pulmonary vein angulation and shortening/straightening the pulmonary vein course may prove effective in surgically managing PVS.
Highlights
Pulmonary vein stenosis (PVS) remains one of the most challenging problems in pediatric heart disease
It is well known that effective management of PVS requires an interdisciplinary team in order to implement multimodal therapy [1]
While an exhaustive account of total anomalous pulmonary venous connection (TAPVC) repair is beyond the scope of this review, for historical context it is worth noting that the surgical correction of TAPVC dates back to the 1950s
Summary
Pulmonary vein stenosis (PVS) remains one of the most challenging problems in pediatric heart disease. Disease was discovered on the left side, where there was localized thickening and stenosis at the junction of a common left pulmonary vein and the left atrium This thickened tissue was incised to widen the stenotic area. Bini and colleagues described several surgical techniques in a small series of eight patients ranging from newborn to four years of age [13] These operations included excision of the stenotic region with direct re-implantation of the healthy pulmonary vein(s) into the left atrium, venoplasty akin to a Heinecke–Mikulicz pyloroplasty (i.e., longitudinal vein incision with transverse closure to widen the stenotic region), the use of prosthetic material (i.e., Gore-Tex®, Dacron®) to widen the vein-atrial junction, and the use of autologous atrial tissue to patch-enlarge the venous pathway. While early surgical innovation aimed to tackle more complex pulmonary vein disease, PVS was still considered a “lethal lesion.”
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
