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Predicting treatment of pulmonary hypertension at discharge in infants with congenital diaphragmatic hernia

Journal of Perinatology volume 42, pages 45–52 (2022)Cite this article

Subjects

Abstract

Objective

To predict pulmonary hypertension (PH) therapy at discharge in a large multicenter cohort of infants with congenital diaphragmatic hernia (CDH).

Study design

Six-year linked records from Children’s Hospitals Neonatal Database and Pediatric Health Information System were used; patients whose diaphragmatic hernia was repaired before admission or referral, who were previously home before admission or referral, and non-survivors were excluded. The primary outcome was the use of PH medications at discharge and the secondary outcome was an inter-center variation of therapies during inpatient utilization. Clinical factors were used to develop a multivariable equation randomly applied to 80% cohort; validated in the remaining 20% infants.

Results

A total of 831 infants with CDH from 23 centers were analyzed. Overall, 11.6% of survivors were discharged on PH medication. Center, duration of mechanical ventilation, and duration of inhaled nitric oxide were associated with the use of PH medication at discharge. This model performed well in the validation cohort area under the receiver operating characteristic curve of 0.9, goodness-of-fit χ2, p = 0.17.

Conclusions

Clinical variables can predict the need for long-term PH medication after NICU hospitalization in surviving infants with CDH. This information may be useful to educate families and guide the development of clinical guidelines.

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Fig. 1: Flow diagram outlining patient selection for the study.
Fig. 2: Intercenter variation of pulmonary hypertension (PH) medication use at any time during NICU stay and at hospital discharge in CDH patients.

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Acknowledgements

Jeanette Asselin, Beverly Brozanski, David Durand (ex officio), Francine Dykes (ex officio), Jacquelyn Evans (Executive Director), Theresa Grover, Karna Murthy (Chair), Michael Padula, Eugenia Pallotto, Anthony Piazza, Kristina Reber, and Billie Short are members of the Children’s Hospitals Neonatal Consortium, Inc. For more information, please contact: support@thechnc.org.

Author information

Authors and Affiliations

  1. UPMC Children’s Hospital of Pittsburgh and the Department of Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA

    Burhan Mahmood

  2. Ann & Robert H Lurie Children’s Hospital of Chicago and the Department of Pediatrics, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA

    Karna Murthy & Nicolas F. M. Porta

  3. Children’s Hospital of Philadelphia and Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA

    Natalie Rintoul & Holly Hedrick

  4. LeBonheur Children’s Hospital and the University of Tennessee Health Science Center, Memphis, TN, USA

    Mark Weems

  5. Children’s Healthcare of Atlanta at Egleston, Emory Children’s Pediatric Institute, and Emory University School of Medicine, Atlanta, GA, USA

    Sarah Keene

  6. St. Louis Children’s Hospital and Department of Pediatrics, Washington University School of Medicine, St. Louis, MO, USA

    Beverly Brozanski

  7. Seattle Children’s Hospital and University of Washington, Seattle, WA, USA

    Robert DiGeronimo

  8. Cincinnati Children’s Hospital Medical Center and University of Cincinnati School of Medicine, Cincinnati, OH, USA

    Beth Haberman

  9. Children’s Hospital Colorado and University of Colorado School of Medicine, Aurora, CO, USA

    Jason Gien & Theresa R. Grover

  10. Nationwide Children’s Hospital, Columbus, OH, USA

    Ruth Seabrook & Kristina Reber

  11. University of Texas Southwestern, Dallas, TX, USA

    Noorjahan Ali

  12. Children’s Hospital Los Angeles and the Fetal & Neonatal Institute, Department of Pediatrics, USC Keck School of Medicine, Los Angeles, CA, USA

    Rachel Chapman

  13. Children’s Mercy Hospitals & Clinics, Department of Pediatrics, University of Missouri Kansas City School of Medicine, Kansas City, MO, USA

    John Daniel

  14. Arkansas Children’s Hospital, Little Rock, AR, USA

    Allen Harrison, Robert Lyle & Becky Rogers

  15. Cook Children’s Hospital, Fort Worth, TX, USA

    Yvette Johnson

  16. Children’s Hospital of Wisconsin and Medical College of Wisconsin, Milwaukee, WI, USA

    Michael Uhing

  17. Children’s Hospital Association, Inc, Lenexa, KS, USA

    Isabella Zaniletti

  18. Children’s Healthcare of Atlanta, Atlanta, GA, USA

    Anthony Piazza

  19. Children’s Healthcare of Atlanta at Scottish Rite, Atlanta, GA, USA

    Gregory Sysyn

  20. Children’s Hospital of Alabama, Birmingham, AL, USA

    Carl Coghill

  21. Le Bonheur Children’s Hospital, Memphis, TN, USA

    Ramasubbareddy Dhanireddy

  22. Children’s Hospital Boston, Boston, MA, USA

    Anne Hansen & Tanzeema Houssain

  23. Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL, USA

    Karna Murthy & Gustave Falciglia

  24. Cincinnati Children’s Hospital, Cincinnati, OH, USA

    Beth Haberman

  25. Children’s Medical Center, Dallas, TX, USA

    Rashmin Savani

  26. Children’s Hospital Colorado, Aurora, CO, USA

    Theresa Grover

  27. Children’s Hospital of Michigan, Detroit, MI, USA

    Girija Natarajan

  28. Cook Children’s Health Care System, Fort Worth, TX, USA

    Annie Chi & Yvette Johnson

  29. Texas Children’s Hospital, Houston, TX, USA

    Gautham Suresh

  30. Riley Children’s Hospital, Indianapolis, IN, USA

    William Engle

  31. Children’s Mercy Hospitals & Clinics, Kansas City, MO, USA

    Eugenia Pallotto

  32. Children’s Hospital Los Angeles and USC Keck School of Medicine, Los Angeles, CA, USA

    Rachel Chapman

  33. American Family Children’s Hospital, Madison, WI, USA

    Jamie Limjoco

  34. Children’s Hospital & Research Center Oakland, Oakland, CA, USA

    Priscilla Joe

  35. The Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Jacquelyn Evans, Michael Padula & David Munson

  36. St. Christopher’s Hospital for Children, Philadelphia, PA, USA

    Suzanne Touch

  37. UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA

    Toby Yanowitz

  38. St. Louis Children’s Hospital, St. Louis, MO, USA

    Beverly Brozanski & Rakesh Rao

  39. All Children’s Hospital, St. Petersburg, FL, USA

    Victor McKay

  40. Rady Children’s Hospital, San Diego, CA, USA

    Mark Speziale & Laurel Moyer

  41. Children’s National Medical Center, Washington, DC, USA

    Billie Short

  42. AI DuPont Hospital for Children, Wilmington, DE, USA

    Kevin Sullivan

  43. Primary Children’s Medical Center, Salt Lake City, UT, USA

    Con Yee Ling

  44. Children’s Hospital of Wisconsin, Milwaukee, WI, USA

    Michael Uhing & Ankur Datta

  45. Children’s Hospital of Omaha, Omaha, NE, USA

    Lynne Willett & Nicole Birge

  46. Florida Hospital for Children, Orlando, FL, USA

    Rajan Wadhawan

  47. Seattle Children’s Hospital, Seattle, WA, USA

    Elizabeth Jacobsen-Misbe & Robert DiGeronimo

  48. Hospital for Sick Children, Toronto, ON, Canada

    Kyong-Soon Lee

  49. Children’s Hospital Orange County, Los Angeles, CA, USA

    Michel Mikhael

Authors
  1. Burhan Mahmood
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  2. Karna Murthy
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  3. Natalie Rintoul
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  4. Mark Weems
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  5. Sarah Keene
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  6. Beverly Brozanski
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  7. Robert DiGeronimo
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  8. Beth Haberman
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  9. Holly Hedrick
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  10. Jason Gien
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  11. Ruth Seabrook
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  12. Noorjahan Ali
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  13. Rachel Chapman
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  14. John Daniel
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  15. Allen Harrison
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  16. Yvette Johnson
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  17. Nicolas F. M. Porta
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  18. Michael Uhing
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  19. Isabella Zaniletti
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  20. Theresa R. Grover
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Consortia

the Children’s Hospitals Neonatal Consortium

  • Anthony Piazza
  • , Gregory Sysyn
  • , Carl Coghill
  • , Ramasubbareddy Dhanireddy
  • , Anne Hansen
  • , Tanzeema Houssain
  • , Karna Murthy
  • , Gustave Falciglia
  • , Beth Haberman
  • , Kristina Reber
  • , Rashmin Savani
  • , Theresa Grover
  • , Girija Natarajan
  • , Annie Chi
  • , Yvette Johnson
  • , Gautham Suresh
  • , William Engle
  • , Eugenia Pallotto
  • , Robert Lyle
  • , Becky Rogers
  • , Rachel Chapman
  • , Jamie Limjoco
  • , Priscilla Joe
  • , Jacquelyn Evans
  • , Michael Padula
  • , David Munson
  • , Suzanne Touch
  • , Toby Yanowitz
  • , Beverly Brozanski
  • , Rakesh Rao
  • , Victor McKay
  • , Mark Speziale
  • , Laurel Moyer
  • , Billie Short
  • , Kevin Sullivan
  • , Con Yee Ling
  • , Michael Uhing
  • , Ankur Datta
  • , Lynne Willett
  • , Nicole Birge
  • , Rajan Wadhawan
  • , Elizabeth Jacobsen-Misbe
  • , Robert DiGeronimo
  • , Kyong-Soon Lee
  •  & Michel Mikhael

Contributions

All authors and members of the Children’s Hospitals Neonatal Consortium Congenital Diaphragmatic Hernia Focus Group conceptualized the study, were responsible for the study design; collection, analysis, and interpretation of the data; and decision to submit the manuscript for publication. BM drafted the initial manuscript, reviewed, and revised the final manuscript. KM, NR, MW, SK, BB, RD, BH, HH, JG, RS, NA, RC, JD, AH, YJ, NFMP, MU, IZ, and TRG critically reviewed and revised the manuscript for important intellectual content. All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work.

Corresponding author

Correspondence to Burhan Mahmood.

Ethics declarations

Competing interests

BB, TRG, and KM are board members of the Children’s Hospitals Neonatal Consortium, 501(c)(3) non-profit organization that manages and owns the Children’s Hospitals Neonatal Database, a data source used in this study. None of the remaining authors have any financial or other conflicts of interest as described in the author’s instructions. There were no study sponsors involved in the creation of the manuscript.

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Mahmood, B., Murthy, K., Rintoul, N. et al. Predicting treatment of pulmonary hypertension at discharge in infants with congenital diaphragmatic hernia. J Perinatol 42, 45–52 (2022). https://doi.org/10.1038/s41372-021-01249-6

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