Pulmonary Vein Stenosis in Children: A Programmatic Approach Employing Primary and Anatomic Therapy

Abstract

Pulmonary vein stenosis (PVS) is a difficult condition to treat due to recurrence and progression. In 2017, we developed a comprehensive PVS Program at our center to address the multidisciplinary needs of these patients. We discuss the components of our program and our approach to these patients, using a combination of primary (medical) therapy in addition to anatomic therapy to preserve vessel patency. A multidisciplinary approach to treating these challenging patients is critical.

Management of Pediatric Pulmonary Vein Stenosis

“…41 Although the decision to treat PVS initially can appear relatively straightforward, it is necessary to explain that the ramifications of entering the "therapeutic pathway" for PVS require a commitment by both the providers and parents. 42 Multivessel PVS therapy includes intensive multimodality treatments, including recurrent invasive interventions, polypharmacy, frequent testing/blood draws, radiation exposure, and prolonged hospitalizations (Central Illustration). Although every patient is unique in their clinical course, characterizing the risk profile for each patient on the basis of studied clinical factors can be helpful when educating the family.…”

Targeted Rapamycin Delivery via Magnetic Nanoparticles to Address Stenosis in a 3D Bioprinted in Vitro Model of Pulmonary Veins

“…[ 1 , 2 ] In the pediatric patients, PVS is often an aggressive disease that is characterized by neointimal obstruction of the PVs, leading to pulmonary hypertension, right heart failure, and death. [ 3 , 4 ] In adults, acquired PVS is increasingly being reported following radiofrequency ablation for atrial fibrillation (1–1.5% incidence rate). [ 5 , 6 ] While post‐ablation PVS typically results in less morbidity and mortality than those reported in the congenital types, these acquired disease could still be highly symptomatic, associated with dyspnea and even hemoptysis.…”