Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Clinical Research Article
  • Published:

Early postnatal echocardiographic characteristics impact survival and extracorporeal life support in congenital diaphragmatic hernia

Pediatric Research (2025)Cite this article

Abstract

Background

Early echocardiographic characteristics (EC) of congenital diaphragmatic hernia (CDH) neonates and their associations with outcomes, especially differences by laterality and size, are unknown.

Methods

Congenital Diaphragmatic Hernia Study Group data between 2015 and 2020 were used. Early postnatal EC, including atrial and ductal shunt direction, pulmonary hypertension (PH) severity, and ventricular size and function, were assessed based on defect laterality and size. Outcomes included mortality and extracoporeal life support (ECLS) use.

Results

The study population included 1777 infants. Severe PH, right-to-left shunt, left ventricular (LV) hypoplasia, right ventricular dilation, and ventricular dysfunction were more prevalent in larger defects. Independent of defect size, neonates with R-CDH had more severe PH, more bidirectional and right-to-left atrial shunt, and more biventricular (BV) dysfunction. In contrast, L-CDH neonates had more LV hypoplasia and left-to-right atrial shunt. After adjusting for defect side, larger defects were associated with LV hypoplasia and right-to-left and bidirectional atrial shunt. In multivariate analysis, right-to-left atrial shunt and BV dysfunction were associated with increased mortality, whereas bidirectional atrial shunt and BV dysfunction were associated with ECLS use.

Conclusions

CDH neonates are at increased risk for early cardiac dysfunction. EC differ by laterality and size. Management of cardiac dysfunction in CDH may improve outcomes.

Impact

  • Cardiac dysfunction has emerged as a factor contributing to adverse outcomes in congenital diaphragmatic hernia (CDH). However, there are limited data on the impact of defect size, laterality, and severity of postnatal cardiac dysfunction on outcomes.

  • Echocardiographic characteristics in the first two days of life differ by defect laterality and size. Right-to-left atrial shunt and biventricular dysfunction are associated with increased mortality. Bidirectional atrial shunt and biventricular dysfunction were associated with extracorporeal life support use.

  • Our results support the need for standardized cardiac function assessment in critically ill neonates with CDH. Future strategies to identify and manage these diverse hemodynamic profiles are needed to improve outcomes.

Access through your institution
Buy or subscribe

This is a preview of subscription content, access via your institution

Access options

Access through your institution

Buy this article

  • Purchase on SpringerLink
  • Instant access to full article PDF
Buy now

Prices may be subject to local taxes which are calculated during checkout

Fig. 1
Fig. 2: The first postnatal echocardiographic characteristics by defect side.
Fig. 3: The first postnatal echocardiographic characteristics by defect size.
Fig. 4: Echocardiographic predictors for mortality and ECLS use based on multivariate regression models.

Similar content being viewed by others

Data availability

The data supporting this study’s findings are available from the CDHSG, but membership restrictions apply. Therefore, the data are not publicly available. Under unique and special circumstances, data could be made available from the authors following reasonable request, for a specific rationale, and with permission of the CDHSG.

References

  1. Morini, F., Lally, P. A., Lally, K. P. & Bagolan, P. The Congenital Diaphragmatic Hernia Study Group registry. Eur. J. Pediatr. Surg. 25, 488–496 (2015).

    Article  PubMed  Google Scholar 

  2. Zani, A. et al. Congenital diaphragmatic hernia. Nat. Rev. Dis. Prim. 8, 37 (2022).

    Article  PubMed  Google Scholar 

  3. Chock, V. Y. et al. In-hospital morbidities for neonates with congenital diaphragmatic hernia: the impact of defect size and laterality. J. Pediatr. 240, 94–101.e106 (2022).

    Article  PubMed  Google Scholar 

  4. Danzer, E. & Hedrick, H. L. Controversies in the management of severe congenital diaphragmatic hernia. Semin Fetal Neonatal Med. 19, 376–384 (2014).

    Article  PubMed  Google Scholar 

  5. Gupta, V. S. et al. Mortality in congenital diaphragmatic hernia: a multicenter registry study of over 5000 patients over 25 years. Ann. Surg. 277, 520–527 (2023).

    Article  PubMed  Google Scholar 

  6. Pammi, M. et al. Prognostic value of echocardiographic parameters in congenital diaphragmatic hernia: a systematic review and meta-analysis. Arch. Dis. Child Fetal Neonatal Ed. 108, 631–637 (2023).

    Article  PubMed  Google Scholar 

  7. Patel, N., Massolo, A. C. & Kipfmueller, F. Congenital diaphragmatic hernia-associated cardiac dysfunction. Semin Perinatol. 44, 151168 (2020).

    Article  PubMed  Google Scholar 

  8. Patel, N. et al. Early postnatal ventricular dysfunction is associated with disease severity in patients with congenital diaphragmatic hernia. J. Pediatr. 203, 400–407.e401 (2018).

    Article  PubMed  Google Scholar 

  9. Altit, G., Bhombal, S., Van Meurs, K. & Tacy, T. A. Ventricular performance is associated with need for extracorporeal membrane oxygenation in newborns with congenital diaphragmatic hernia. J. Pediatr. 191, 28–34.e21 (2017).

    Article  PubMed  Google Scholar 

  10. Patel, N. et al. Ventricular dysfunction is a critical determinant of mortality in congenital diaphragmatic hernia. Am. J. Respir. Crit. Care Med. 200, 1522–1530 (2019).

    Article  PubMed  Google Scholar 

  11. Dao, D. T. et al. Early left ventricular dysfunction and severe pulmonary hypertension predict adverse outcomes in “low-risk” congenital diaphragmatic hernia. Pediatr. Crit. Care Med. 21, 637–646 (2020).

    Article  PubMed  PubMed Central  Google Scholar 

  12. Johng, S. et al. Unique Cardiopulmonary interactions in congenital diaphragmatic hernia: physiology and therapeutic implications. Neoreviews 24, e720–e732 (2023).

    Article  PubMed  Google Scholar 

  13. Tsao, K. & Lally, K. P. The Congenital Diaphragmatic Hernia Study Group: a voluntary international registry. Semin Pediatr. Surg. 17, 90–97 (2008).

    Article  PubMed  Google Scholar 

  14. Harting, M. T. & Lally, K. P. The Congenital Diaphragmatic Hernia Study Group Registry update. Semin Fetal Neonatal Med. 19, 370–375 (2014).

    Article  PubMed  Google Scholar 

  15. Noh, C. Y. et al. Early Nitric oxide is not associated with improved outcomes in congenital diaphragmatic hernia. Pediatr. Res. 93, 1899–1906 (2023).

    Article  CAS  PubMed  Google Scholar 

  16. Ferguson, D. M. et al. Early, Postnatal pulmonary hypertension severity predicts inpatient outcomes in congenital diaphragmatic hernia. Neonatology 118, 147–154 (2021).

    Article  PubMed  Google Scholar 

  17. Kinsella, J. P. et al. The Left ventricle in congenital diaphragmatic hernia: implications for the management of pulmonary hypertension. J. Pediatr. 197, 17–22 (2018).

    Article  PubMed  Google Scholar 

  18. Wehrmann, M. et al. Implications of atrial-level shunting by echocardiography in newborns with congenital diaphragmatic hernia. J. Pediatr. 219, 43–47 (2020).

    Article  PubMed  Google Scholar 

  19. Lally, K. P. et al. Standardized reporting for congenital diaphragmatic hernia—an international consensus. J. Pediatr. Surg. 48, 2408–2415 (2013).

    Article  PubMed  Google Scholar 

  20. Congenital Diaphragmatic Hernia Study, G. et al. Defect size determines survival in infants with congenital diaphragmatic hernia. Pediatrics 120, e651–e657 (2007).

    Article  Google Scholar 

  21. Prasad, R., Saha, B. & Kumar, A. Ventricular function in congenital diaphragmatic hernia: a systematic review and meta-analysis. Eur. J. Pediatr. 181, 1071–1083 (2022).

    Article  PubMed  Google Scholar 

  22. Moenkemeyer, F. & Patel, N. Right ventricular diastolic function measured by tissue doppler imaging predicts early outcome in congenital diaphragmatic hernia. Pediatr. Crit. Care Med. 15, 49–55 (2014).

    Article  PubMed  Google Scholar 

  23. Le, L. S., Kinsella, J. P., Gien, J. & Frank, B. S. Failure to normalize biventricular function is associated with extracorporeal membrane oxygenation use in neonates with congenital diaphragmatic hernia. J. Pediatr. 260, 113490 (2023).

    Article  PubMed  Google Scholar 

  24. Fraga, M. V. et al. Congenital diaphragmatic hernia patients with left heart hypoplasia and left ventricular dysfunction have highest odds of mortality. J. Pediatr. 271, 114061 (2024).

    Article  PubMed  PubMed Central  Google Scholar 

  25. Toyoshima, K. et al. Right to left ventricular volume ratio is associated with mortality in congenital diaphragmatic hernia. Pediatr. Res. 94, 304–312 (2023).

    Article  PubMed  PubMed Central  Google Scholar 

  26. Vogel, M. et al. Significance and outcome of left heart hypoplasia in fetal congenital diaphragmatic hernia. Ultrasound Obstet. Gynecol. 35, 310–317 (2010).

    Article  CAS  PubMed  Google Scholar 

  27. Pelizzo, G. et al. Cardiac adaptation to severe congenital diaphragmatic hernia. Fetal Pediatr. Pathol. 35, 10–20 (2016).

    Article  PubMed  Google Scholar 

  28. Karamanoukian, H. L. et al. Pathophysiology of congenital diaphragmatic hernia. Xi: anatomic and biochemical characterization of the heart in the fetal lamb Cdh model. J. Pediatr. Surg. 30, 925–928 discussion 929 (1995).

    Article  CAS  PubMed  Google Scholar 

  29. Van Mieghem, T. et al. Left ventricular cardiac function in fetuses with congenital diaphragmatic hernia and the effect of fetal endoscopic tracheal occlusion. Ultrasound Obstet. Gynecol. 34, 424–429 (2009).

    Article  PubMed  Google Scholar 

  30. Baumgart, S. et al. Cardiac malposition, redistribution of fetal cardiac output, and left heart hypoplasia reduce survival in neonates with congenital diaphragmatic hernia requiring extracorporeal membrane oxygenation. J. Pediatr. 133, 57–62 (1998).

    Article  CAS  PubMed  Google Scholar 

  31. Cruz-Lemini, M. et al. Characterizing cardiac dysfunction in fetuses with left congenital diaphragmatic hernia. Prenat. Diagn. 38, 422–427 (2018).

    Article  CAS  PubMed  Google Scholar 

  32. DeKoninck, P., D’Hooge, J., Van Mieghem, T., Richter, J. & Deprest, J. Speckle tracking echocardiography in fetuses diagnosed with congenital diaphragmatic hernia. Prenat. Diagn. 34, 1262–1267 (2014).

    Article  PubMed  Google Scholar 

  33. Kosiv, K. A. et al. Fetal cerebrovascular impedance is reduced in left congenital diaphragmatic hernia. Ultrasound Obstet. Gynecol. 57, 386–391 (2021).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  34. Holder, A. M. et al. Genetic factors in congenital diaphragmatic hernia. Am. J. Hum. Genet 80, 825–845 (2007).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  35. Cannata, G., Caporilli, C., Grassi, F., Perrone, S. & Esposito, S. Management of congenital diaphragmatic hernia (Cdh): role of molecular genetics. Int. J. Mol. Sci. 22 (2021).

  36. Zhaorigetu, S., Gupta, V. S., Jin, D. & Harting, M. T. Cardiac energy metabolism may play a fundamental role in congenital diaphragmatic hernia-associated ventricular dysfunction. J. Mol. Cell Cardiol. 157, 14–16 (2021).

    Article  CAS  PubMed  Google Scholar 

  37. Patel, N., Massolo, A. C., Kraemer, U. S. & Kipfmueller, F. The heart in congenital diaphragmatic hernia: knowns, unknowns, and future priorities. Front Pediatr. 10, 890422 (2022).

    Article  PubMed  PubMed Central  Google Scholar 

  38. Altit, G., Lapointe, A., Kipfmueller, F. & Patel, N. Cardiac function in congenital diaphragmatic hernia. Semin Pediatr. Surg. 33, 151438 (2024).

    Article  PubMed  Google Scholar 

  39. Lefebvre, C. et al. Feasibility and safety of intact cord resuscitation in newborn infants with congenital diaphragmatic hernia (Cdh). Resuscitation 120, 20–25 (2017).

    Article  PubMed  Google Scholar 

  40. Kashyap, A. J. et al. Neonatal cardiopulmonary transition in an ovine model of congenital diaphragmatic hernia. Arch. Dis. Child Fetal Neonatal Ed. 104, F617–F623 (2019).

    Article  PubMed  Google Scholar 

  41. DeKoninck, P. L. J. et al. Effects of tracheal occlusion on the neonatal cardiopulmonary transition in an ovine model of diaphragmatic hernia. Arch. Dis. Child Fetal Neonatal Ed. 104, F609–F616 (2019).

    Article  PubMed  Google Scholar 

Download references

Acknowledgements

We acknowledge the ongoing contributions of highly committed Congenital Diaphragmatic Hernia Study Group member centers that voluntarily participate in studying congenital diaphragmatic hernia. This study was supported by a grant from the Maternal & Child Health Research Institute at Stanford awarded to C.Y.N. The sponsor had no role in the design and conduct of the study, the collection, management, analysis, and interpretation of the data, the preparation, review, or approval of the manuscript, or the decision to submit the manuscript for publication.

Author information

Author notes

  1. A list of members and their affiliations appears in the Supplementary Information.

  2. These authors contributed equally: Caroline Y. Noh, Enrico Danzer.

Authors and Affiliations

  1. Division of Neonatology, Fetal and Neonatal Institute, Children’s Hospital Los Angeles, University of Southern California Keck School of Medicine, Los Angeles, CA, USA

    Caroline Y. Noh

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

    Enrico Danzer, Valerie Y. Chock & Krisa P. Van Meurs

  3. Department of Surgery, Division of Pediatric Surgery, Memorial Sloan Kettering Cancer Center, Weill Cornell Medical College, New York, NY, USA

    Enrico Danzer

  4. Children’s Healthcare of Atlanta, Emory University, Atlanta, GA, USA

    Shazia Bhombal

  5. Royal Hospital for Children Glasgow, Glasgow, UK

    Neil Patel

  6. Department of Biostatistics, New York University, New York, NY, USA

    Alex Dahlen

  7. Department of Pediatric Surgery, McGovern Medical School at the University of Texas Health Science Center at Houston and Children’s Memorial Hermann Hospital, Houston, TX, USA

    Matthew T. Harting, Kevin P. Lally & Ashley H. Ebanks

Authors
  1. Caroline Y. Noh
    View author publications

    Search author on:PubMed Google Scholar

  2. Enrico Danzer
    View author publications

    Search author on:PubMed Google Scholar

  3. Shazia Bhombal
    View author publications

    Search author on:PubMed Google Scholar

  4. Valerie Y. Chock
    View author publications

    Search author on:PubMed Google Scholar

  5. Neil Patel
    View author publications

    Search author on:PubMed Google Scholar

  6. Alex Dahlen
    View author publications

    Search author on:PubMed Google Scholar

  7. Matthew T. Harting
    View author publications

    Search author on:PubMed Google Scholar

  8. Kevin P. Lally
    View author publications

    Search author on:PubMed Google Scholar

  9. Ashley H. Ebanks
    View author publications

    Search author on:PubMed Google Scholar

  10. Krisa P. Van Meurs
    View author publications

    Search author on:PubMed Google Scholar

Consortia

for the Congenital Diaphragmatic Hernia Study Group

Contributions

C.Y.N., E.D., S.B., V.Y.C., and K.P.V.M. contributed substantially to the study’s conception and design, acquisition, analysis, and interpretation of data, and drafting and critical revision of the manuscript. N.P., A.D., M.T.H., K.P.L., and A.H.E. contributed substantially to the acquisition, analysis, and interpretation of data and critical revision of the manuscript for important intellectual content. All the authors agreed and approved this version to be submitted for publication.

Corresponding author

Correspondence to Enrico Danzer.

Ethics declarations

Competing interests

The authors declare no competing interests.

Consent to participate

The CDHSG registry is hosted by the University of Texas at Houston and approved by its Institutional Review Board (HSC-MS-03-223) with a waiver of informed consent

Additional information

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

Supplementary information

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Noh, C.Y., Danzer, E., Bhombal, S. et al. Early postnatal echocardiographic characteristics impact survival and extracorporeal life support in congenital diaphragmatic hernia. Pediatr Res (2025). https://doi.org/10.1038/s41390-025-04443-w

Download citation

  • Received:

  • Accepted:

  • Published:

  • DOI: https://doi.org/10.1038/s41390-025-04443-w

Search

Advanced search

Quick links