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Death or resolution: the “natural history” of pulmonary hypertension in bronchopulmonary dysplasia

Journal of Perinatology volume 39, pages 415–425 (2019)Cite this article

Subjects

Abstract

Objectives

The primary objective was to describe the early “natural history” of pulmonary hypertension (PH) in the premature population. The secondary objective was to describe factors associated with poor outcomes in the premature population with PH at 36 weeks post-menstrual age (PMA).

Study design

Retrospective chart review of patients followed at our institution from 2000 to 2017 with echocardiographic (ECHO) evidence of PH at 36 weeks PMA, and born ≤ 32 weeks estimated gestational age (GA). Cox regression was used for survival analysis.

Results

Sixty-one patients with PH (26.5 ± 1.5 weeks at birth) were included. All PH patients had bronchopulmonary dysplasia (BPD), with 89% considered severe; 38% were small for gestational age. Necrotizing enterocolitis requiring surgery was common (25%). Use of post-natal steroids (HR 11.02, p = 0.01) and increased severity of PH (HR 1.05, p < 0.001) were associated with mortality. Pulmonary vein stenosis (PVS) was documented in 26% of the PH cohort, but not associated with increased mortality. ECHO estimation of pulmonary artery pressure (PAP) was available in 84%. PAP was higher in those who died (sPAP/sBP ratio 1.09 ± 27 vs 0.83 ± 20 %, p = 0.0002). At follow-up (mean 250 ± 186 weeks PMA), 72% of the PH cohort was alive. Most survivors (66%) had resolution of their PH on their most recent ECHO; 31% remained on PH therapy.

Conclusion

PH resolved in most survivors in this study population. Mortality in those with BPD-PH was associated with male sex, post-natal steroid use, and increased severity of PH, but not with PVS.

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Acknowledgements

We acknowledge Dr. Gautier Gabriel Marcq, MD, MSc, for his review of the statistical analysis.

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Authors and Affiliations

  1. Division of Neonatology - Department of Pediatrics - McGill University, Montreal Children’s Hospital – McGill University Health Centre, Montreal, Canada

    Gabriel Altit

  2. Neonatal and Developmental Medicine, Stanford University School of Medicine – Lucile Packard Children’s Hospital, Stanford, CA, USA

    Shazia Bhombal

  3. Pediatric Cardiology, Stanford University School of Medicine - Lucile Packard Children’s Hospital, Stanford, Stanford, CA, USA

    Rachel K. Hopper, Theresa A. Tacy & Jeffrey Feinstein

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  1. Gabriel Altit
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Contributions

Gabriel Altit designed the protocol, worked on the data collection and analysis, and prepared the manuscript. Shazia Bhombal and Theresa Tacy helped in the design of the study and on the preparation of the manuscript. Rachel Hopper helped in the interpretation of data and on preparation of manuscript. Jeffrey Feinstein overviewed the whole process from design of the study to preparation of final manuscript.

Corresponding author

Correspondence to Gabriel Altit.

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Conflict of interest

The authors declare no conflicts of interest related to the content of this study. Gabriel Altit is the author that wrote the first draft. This study was approved by the institutional review board of Stanford University (protocol - IRB-39388). The study was supported by the Vera Moulton Wall Center for Pulmonary Vascular Disease at Stanford.

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Altit, G., Bhombal, S., Hopper, R.K. et al. Death or resolution: the “natural history” of pulmonary hypertension in bronchopulmonary dysplasia. J Perinatol 39, 415–425 (2019). https://doi.org/10.1038/s41372-018-0303-8

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