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Treatment of pulmonary hypertension during initial hospitalization in a multicenter cohort of infants with congenital diaphragmatic hernia (CDH)

Abstract

Objective

Describe inpatient pulmonary hypertension (PH) treatment and factors associated with therapy at discharge in a multicenter cohort of infants with CDH.

Methods

Six years linked records from Children’s Hospitals Neonatal Database and Pediatric Health Information System were used to describe associations between prenatal/perinatal factors, clinical outcomes, echocardiographic findings and PH medications (PHM), during hospitalization and at discharge.

Results

Of 1106 CDH infants from 23 centers, 62.8% of infants received PHM, and 11.6% of survivors were discharged on PHM. Survivors discharged on PHM more frequently had intrathoracic liver, small for gestational age, and low 5 min APGARs compared with those discharged without PHM (p < 0.0001). Nearly one-third of infants discharged without PHM had PH on last inpatient echo.

Conclusions

PH medication use is common in CDH. Identification of infants at risk for persistent PH may impact ongoing management. Post-discharge follow-up of all CDH infants with echocardiographic evidence of PH is warranted.

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Fig. 1: Percentage of patients treated with each medication by year.

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Data availability

The data set used for this study is derived from the Children’s Hospital Neonatal Database (CHND), and is not publicly available due to data use agreement restrictions from the member institutions. Data are however available from the authors upon reasonable request and with permission of the CHND.

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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.

We are indebted to the following institutions that serve the infants and their families, and these institutions also have invested in and continue to participate in the Children’s Hospital’s Neonatal Database (CHND). The site sponsors/contributors for the CHND are also included: Anthony Piazza, Children’s Healthcare of Atlanta, Atlanta, GA; Gregory Sysyn, Children’s Healthcare of Atlanta at Scottish Rite; Carl Coghill, Children’s Hospital of Alabama, Birmingham, AL; Ajay Talati, Le Bonheur Children’s Hospital, Memphis, TN; Anne Hansen, Tanzeema Houssain, Children’s Hospital Boston, Boston, MA; Karna Murthy, Gustave Falciglia, Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL; Beth Haberman, Cincinnati Children’s Hospital, Cincinnati, OH; Kristina Reber, Nationwide Children’s Hospital, Columbus, OH; Rashmin Savani, Children’s Medical Center, Dallas, TX; Theresa Grover, Children’s Hospital Colorado, Aurora, CO; Girija Natarajan, Children’s Hospital of Michigan, Detroit, MI; Annie Chi, Yvette Johnson, Cook Children’s Health Care System, Fort Worth, TX; Gautham Suresh, Texas Children’s Hospital, Houston, TX; William Engle, Riley Children’s Hospital, Indianapolis, IN; Eugenia Pallotto, Children’s Mercy Hospitals & Clinics, Kansas City, MO; Robert Lyle, Becky Rogers, Arkansas Children’s Hospital, Little Rock, AR; Rachel Chapman, Children’s Hospital Los Angeles, Los Angeles, CA; Jamie Limjoco, American Family Children’s Hospital, Madison, WI; Priscilla Joe, Children’s Hospital & Research Center Oakland, Oakland, CA; Jacquelyn Evans, Michael Padula, David Munson, The Children’s Hospital of Philadelphia, Philadelphia, PA; Suzanne Touch, St. Christopher’s Hospital for Children, Philadelphia, PA; Beverly Brozanski, Children’s Hospital of Pittsburgh of UPMC, Pittsburgh, PA; Rakesh Rao, Amit Mathur, St. Louis Children’s Hospital, St Louis, MO; Victor McKay, All Children’s Hospital, St. Petersburg, FL; Mark Speziale, Laurel Moyer, Rady Children’s Hospital, San Diego, CA; Billie Short, Children’s National Medical Center, Washington, DC; Kevin Sullivan, AI DuPont Hospital for Children, Wilmington, DE; Con Yee Ling, Primary Children’s Medical Center, Salt Lake City, UT; Michael Uhing, Ankur Datta, Children’s Hospital of Wisconsin, Milwaukee, WI; Lynne Willett, Nicole Birge, Children’s Hospital of Omaha; Rajan Wadhawan, Florida Hospital for Children; Elizabeth Jacobsen-Misbe, Robert DiGeronimo, Seattle Children’s Hospital, Seattle, WA; Kyong-Soon Lee, Hospital for Sick Children, Toronto, ON; Michel Mikhael, Children’s Hospital Orange County, Los Angeles, CA.

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Contributions

RBS helped design the study, analyze data, and drafted the initial manuscript. TRG, NR, MW, SK, BH, HH, JG, HAH, NFMP, and KM helped design the study, analyze data, and reviewed and edited manuscript. BB, RD, and NA helped conceptualize the study and reviewed and edited manuscript. RC helped design the study and reviewed and edited the manuscript. JD helped analyze data and reviewed and edited the manuscript. YJ and MU helped conceptualize the study and reviewed and edited the manuscript. IZ performed primary data curation and analysis, and reviewed and edited the manuscript.

Corresponding author

Correspondence to Ruth B. Seabrook.

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Conflict of interest

BB, TG, 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 instructions.

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Publisher’s note Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Members of the Children’s Hospitals Neonatal Consortium Congenital Diaphragmatic Hernia Focus Group are listed below acknowledgements.

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Seabrook, R.B., Grover, T.R., Rintoul, N. et al. Treatment of pulmonary hypertension during initial hospitalization in a multicenter cohort of infants with congenital diaphragmatic hernia (CDH). J Perinatol 41, 803–813 (2021). https://doi.org/10.1038/s41372-021-00923-z

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