David B. Frank, Philip T. Levy, Corey A. Stiver, Brian A. Boe, Christopher W. Baird, Ryan M. Callahan, Charles V. Smith, Rachel D. Vanderlaan, Carl H. Backes
University of Pennsylvania and Children’s Hospital of Philadelphia. Harvard University and Boston Children’s Hospital. Ohio State University and Nationwide Children’s Hospital. University of Washington and Seattle Children’s Research Institute. Columbus University and New York Presbyterian Morgan Stanley Children’s Hospital. University of Toronto and Hospital for Sick Children
United States and Canada
Journal of Perinatology
J Perinatol 2021; 41: 1528-1539
DOI: 10.1038/s41372-021-01008-7
Abstract
Primary pulmonary vein stenosis (PPVS) is an emerging problem among infants. In contrast to acquired disease, PPVS is the development of stenosis in the absence of preceding intervention. While optimal care approaches remain poorly characterized, over the past decade, understanding of potential pathophysiological mechanisms and development of novel therapeutic strategies are increasing. A multidisciplinary team of health care providers was assembled to review the available evidence and provide a common framework for the diagnosis, management, and treatment of PPVS during infancy. To address knowledge gaps, institutional and multi-institutional approaches must be employed to generate knowledge specific to ex-premature infants with PPVS. Within individual institutions, creation of a team comprised of dedicated health care providers from diverse backgrounds is critical to accelerate clinical learning and provide care for infants with PPVS. Multi-institutional collaborations, such as the PVS Network, provide the infrastructure and statistical power to advance knowledge for this rare disease.
Category
Stenosis or Obstruction of Normal Pulmonary Venous Connections
Multidisciplinary Care
Year of Publication: 2021
Age Focus: Pediatric
Article Type: Review
Article Access: Free PDF File or Full Text Article Available Through PubMed or DOI: No