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Factors associated with discontinuation of pulmonary vasodilator therapy in children with bronchopulmonary dysplasia-associated pulmonary hypertension

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Abstract

Objective

To evaluate factors associated with discontinuation of pulmonary vasodilator therapy in bronchopulmonary dysplasia-related pulmonary hypertension (BPD-PH).

Study design

Retrospective study of neonatal, echocardiographic, and cardiac catheterization data in 121 infants with BPD-PH discharged on pulmonary vasodilator therapy from 2009–2020 and followed into childhood.

Result

After median 4.4 years, medications were discontinued in 58%. Those in whom medications were discontinued had fewer days of invasive support, less severe BPD, lower incidence of PDA closure or cardiac catheterization, and higher incidence of fundoplication or tracheostomy decannulation (p < 0.05). On multivariable analysis, likelihood of medication discontinuation was lower with longer period of invasive respiratory support [HR 0.95 (CI:0.91–0.99), p = 0.01] and worse RV dilation on pre-discharge echocardiogram [HR 0.13 (CI:0.03–0.70), p = 0.017]. In those with tracheostomy, likelihood of medication discontinuation was higher with decannulation [HR 10.78 (CI:1.98–58.59), p < 0.001].

Conclusion

In BPD-PH, childhood discontinuation of pulmonary vasodilator therapy is associated with markers of disease severity.

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Fig. 1: The Kaplain–Meier curves for time to pulmonary vasodilator discontinuation are presented.

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Acknowledgements

This study was supported by the Children’s Hospital of Philadelphia Divisions of Cardiology and Neonatology.

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Authors and Affiliations

  1. Division of Cardiology, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Catherine M. Avitabile, Yan Wang, Devon Ash, Laura Mercer-Rosa & David B. Frank

  2. Echocardiography Laboratory Research Unit, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Catherine M. Avitabile, Yan Wang, Devon Ash & Laura Mercer-Rosa

  3. Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA

    Catherine M. Avitabile, Laura Mercer-Rosa, Julie L. Fierro, David B. Frank & Kathleen A. Gibbs

  4. Data Science and Biostatistics Unit, Department of Biomedical and Health Informatics, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Xuemei Zhang & Steve B. Ampah

  5. Division of Neonatology, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Kathleen Nilan & Kathleen A. Gibbs

  6. Division of Pulmonary and Sleep Medicine, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Julie L. Fierro

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Contributions

CMA conceptualized the project, supervised the acquisition of data, performed formal analyses, and wrote the manuscript. XZ and SA developed the statistical methodology and performed formal analyses. YW and DA performed echocardiographic analyses. KN helped conceptualize the project and supervised the acquisition of data. LMR helped conceptualize the project, developed methodology, and performed formal analyses. JLF and DBF helped conceptualize the project. KAG helped conceptualize the project, supervised the acquisition of data, and provided resources. All authors reviewed and/or edited the final manuscript.

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Correspondence to Catherine M. Avitabile.

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Avitabile, C.M., Zhang, X., Ampah, S.B. et al. Factors associated with discontinuation of pulmonary vasodilator therapy in children with bronchopulmonary dysplasia-associated pulmonary hypertension. J Perinatol 42, 1246–1254 (2022). https://doi.org/10.1038/s41372-022-01421-6

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