Jennifer Schramm, Sivakumar Sivalingam, Guillermo E. Moreno, Dinh Quang Le Thanh, Kimberlee Gauvreau, Kaitlin Doherty-Schmeck, Kathy J. Jenkins
Children’s National Hospital. National Heart Institute. Hospital de Pediatría “Professor Dr. Juan P. Garrahan”. Children’s Hospital 1. Boston Children’s Hospital, Boston.
United States, Malaysia, Argentina, Vietnam
Children
Children 2021; 8
DOI: 10.3390/children8030198
Abstract
Pulmonary vein stenosis (PVS) is a rare, but high mortality and resource intensive disease caused by mechanical obstruction or intraluminal myofibroproliferation, which can be post-surgical or idiopathic. There are increasing options for management including medications, cardiac catheterization procedures, and surgery. We queried the International Quality Improvement Collaborative for Congenital Heart Disease (IQIC) database for cases of PVS and described the cohort including additional congenital lesions and surgeries as well as infectious and mortality outcomes. IQIC is a quality improvement project in low-middle-income countries with the goal of reducing mortality after congenital heart surgery. Three cases were described in detail with relevant images. We identified 57 cases of PVS surgery, with similar mortality to higher income countries. PVS should be recognized as a global disease. More research and collaboration are needed to understand the disease, treatments, and outcomes, and to devise treatment approaches for low resource environments.
Category
Stenosis or Obstruction of Normal Pulmonary Venous Connections
Stenosis or Obstruction of Pulmonary Veins Following Surgical Repair of Anomalous Pulmonary Venous Connections
Length of Life Associated with Pulmonary Venous Obstruction
Year of Publication: 2021
Age Focus: Pediatric
Article Type: Literature Review and Analysis
Article Access: Free PDF File or Full Text Article Available Through PubMed or DOI: Yes