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Primary pulmonary vein stenosis among premature infants with single-vessel disease

Journal of Perinatology volume 41, pages 1621–1626 (2021)Cite this article

Subjects

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.

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Fig. 1: Patient selection flowchart.
Fig. 2: Kaplan–Meier survival plot among infants with PPVS disease progression or disease stabilization.

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Funding

The authors (CB, JS, and BC) are funded by the National Heart, Lung, and Blood Institute of the US National Institutes of Health (R01HL145032, R01HL13693). However, this funding is unrelated to the topic of this article and did not contribute to this work product.

Author information

Authors and Affiliations

  1. Center for Perinatal Research, The Abigail Wexner Research Institute at Nationwide Children’s Hospital, Columbus, OH, USA

    Eli Zettler, Brian K. Rivera, Jonathan L. Slaughter, Bernadette Chen & Carl H. Backes

  2. The Heart Center at Nationwide Children’s Hospital, Columbus, OH, USA

    Corey Stiver, Brian Boe, Clifford Cua & Carl H. Backes

  3. Division of Neonatology, The Ohio State University Wexner Medical Center, Columbus, OH, USA

    Molly K. Ball, Jonathan L. Slaughter, Bernadette Chen & Carl H. Backes

  4. Seattle Children’s Research Institute, University of Washington School of Medicine, Seattle, WA, USA

    Charles V. Smith

  5. Department of Pediatrics, The Ohio State University College of Medicine, Columbus, OH, USA

    Jonathan L. Slaughter, Bernadette Chen & Carl H. Backes

  6. Division of Epidemiology, The Ohio State University College of Public Health, Columbus, OH, USA

    Jonathan L. Slaughter

  7. Department of Cardiology, Boston Children’s Hospital and Harvard Medical School, Boston, MA, USA

    Ryan Callahan

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  1. Eli Zettler
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Correspondence to Carl H. Backes.

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Zettler, E., Rivera, B.K., Stiver, C. et al. Primary pulmonary vein stenosis among premature infants with single-vessel disease. J Perinatol 41, 1621–1626 (2021). https://doi.org/10.1038/s41372-020-00830-9

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