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. 2021 Jul;41(7):1621-1626.
doi: 10.1038/s41372-020-00830-9. Epub 2020 Sep 25.

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

Eli Zettler  1 , Brian K Rivera  1 , Corey Stiver  2 , Brian Boe  2 , Clifford Cua  2 , Molly K Ball  3 , Charles V Smith  4 , Jonathan L Slaughter  1   3   5   6 , Bernadette Chen  1   3   5 , Ryan Callahan  7 , Carl H Backes  8   9   10   11
Affiliations

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

Eli Zettler et al. J Perinatol. 2021 Jul.

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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Conflict of interest statement

Conflict of Interest Statement: The authors have no commercial or financial conflicts of interest to disclose.

Figures

Figure 1:
Figure 1:
Patient selection flowchart.
Figure 2:
Figure 2:
Kaplan-Meier survival plot among infants with PPVS disease progression or disease stabilization. Survival is shown on the y-axis. Follow-up time in months is shown on the X-axis. Difference in survival was determined by log-rank test. P-value <0.01.
See this image and copyright information in PMC

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