Eli Zettler, Brian K. Rivera, Corey Stiver, Brian Boe, Clifford Cua, Molly K. Ball, Charles V. Smith, Jonathan L. Slaughter, Bernadette Chen, Ryan Callahan, Carl H. Backes
Nationwide Children’s Hospital and Ohio State University. University of Washington School of Medicine. Boston Children’s Hospital and Harvard Medical School.
United States
Journal of Perinatology
J Perinatol 2021; 41: 1621-1626
DOI: 10.1038/s41372-020-00830-9
Abstract
Objectives: Describe outcomes among preterm infants diagnosed with single-vessel primary pulmonary vein stenosis (PPVS) initially treated using conservative management (active surveillance with deferral of treatment).
Study design: Retrospective cohort study at a single, tertiary-center (2009-2019) among infants <37 weeks’ gestation with single-vessel PPVS. Infants were classified into two categories: disease progression and disease stabilization. Cardiopulmonary outcomes were examined, and a Kaplan-Meier survival analysis performed.
Results: Twenty infants were included. Compared to infants in the stable group (0/10, 0%), all infants in the progressive group had development of at least severe stenosis or atresia (10/10, 100%; P < 0.01). Severe pulmonary hypertension at diagnosis was increased in the progressive (5/10, 50%) versus the stable group (0/10, 0%; P = 0.03). Survival was lower among infants in the progressive than the stable group (log-rank test, P < 0.01).
Conclusion: Among preterm infants with single-vessel PPVS, risk stratification may be possible, wherein more targeted, individualized therapies could be applied.
Category
Stenosis or Obstruction of Normal Pulmonary Venous Connections
Pulmonary Hypertension Associated with Stenosis or Atresia of Pulmonary Veins
Length of Life Associated with Pulmonary Venous Obstruction
Diagnostic Testing. Risk Stratification
Year of Publication: 2021
Age Focus: Pediatric
Article Type: Retrospective Observational Cohort Studies (>10 patients)
Article Access: Free PDF File or Full Text Article Available Through PubMed or DOI: Yes