Carl H. Backes, Erin Nealon, Aimee K. Armstrong, Clifford L. Cua, Courtney Mitchell, Usha Krishnan, Rachel D. Vanderlaan, Mi Kyoung Song, Nicola Viola, Charles V. Smith, Patrick I. McConnell, Brian K. Rivera, Jeffrey Bridge
Nationwide Children’s Hospital and The Ohio State University. Columbia University. University of Toronto and Hospital for Sick Children. Seoul National University Children’s Hospital. University Hospital of Southampton and Children’s Hospital of Southampton. University of Washington School of Medicine.
United States, Canada, Republic of Korea and United Kingdom
Journal of Pediatrics
J Pediatr 2018; 198: 36-45
DOI: 10.1016/j.jpeds.2018.02.030
Abstract
Objective: To quantify outcomes of infants (<1 year of age) diagnosed with pulmonary vein stenosis (PVS).
Study design: MEDLINE (PubMed), Scopus, and Web of Science were searched through February 1, 2017, with no language restrictions. Publications including infants diagnosed with primary PVS, defined as the absence of preceding intervention(s), were considered. The study was performed according to Meta-analysis of Observational Studies in Epidemiology guidelines, the Systematic Reviews, and Meta-Analysis checklist, and registered prospectively. The quality of selected reports was critically examined. Data extraction was independently performed by multiple observers with outcomes agreed upon a priori. Data were pooled using an inverse variance heterogeneity model with incidence of mortality the primary outcome of interest.
Results: Forty-eight studies of 185 infants were included. Studies were highly diverse with regards to the participants, interventions, and outcomes reported. The median (range) age at diagnosis was 5.0 (0.1-11.6) months. Pooled mortality was 58.5% (95% CI 49.8%-67.0%, I2 = 21.4%). We observed greater mortality incidence among infants with 3 or 4 vein stenoses than in those with 1 or 2 vein stenoses (83.3% vs 36.1%; P < .01). We observed greater mortality among infants with bilateral than unilateral disease (78.7% vs 26.0%; P < .01).
Conclusions: Studies of primary PVS during infancy are highly variable in their methodological quality and estimates of clinical outcomes; therefore, estimates of prognosis remain uncertain. Multicenter, interdisciplinary collaborations, including alignment of key outcome measurements, are needed to answer questions beyond the scope of available data.
Category
Stenosis or Obstruction of Normal Pulmonary Venous Connections
Incidence or Prevalence of Disease
Length of Life Associated with Pulmonary Venous Obstruction
Year of Publication: 2018
Age Focus: Pediatric
Article Type: Literature Review and Analysis
Article Access: Free PDF File or Full Text Article Available Through PubMed or DOI: No