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Fetal cardiac dimensions in congenital diaphragmatic hernia: relationship with gestational age and postnatal outcomes

Journal of Perinatology volume 41, pages 1651–1659 (2021)Cite this article

Subjects

Abstract

Objective:

To serially assess fetal cardiac dimensions in congenital diaphragmatic hernia (CDH) and their relation to disease severity.

Study design:

Retrospective analysis of CDH cases and matched controls. Mitral (MVd) and tricuspid (TVd) valve diameters, left (LV) and right (RV) ventricular length and area, Z-scores, were serially assessed at 24–26, 30–32, and 35–37 weeks gestational age (GA).

Results:

In CDH cases MVd, MVd Z-score, and LV area were significantly reduced at 24–26 and 35–37 weeks GA. TVd, TVd Z-score, and RV area were significantly reduced at 24–26 weeks. RV area Z-score increased with advancing GA. MVd and MVd Z-score were significantly lower at 24–26 weeks GA in CDH who had a combined outcome of death and/or ECMO.

Conclusions:

LV hypoplasia in CDH is characterized by reduced MVd from 24 weeks GA. MVd, and the ratio of mitral and tricuspid valve diameters at later gestations, may be potential predictors of disease severity.

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Fig. 1: Fetal cardiac dimensions at three gestational time periods in CDH cases and Controls.
Fig. 2: Mitral valve dimensions at 24–26 weeks gestation and outcome in CDH cases.
Fig. 3: Representation of changes in fetal cardiac dimensions in CDH and control infants, at the three gestational time periods.

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References

  1. Harting MT, Lally KP. The Congenital Diaphragmatic Hernia Study Group registry update. Semin Fetal Neonatal Med. 2014;19:370–5.

    Article  Google Scholar 

  2. Harting MT. Congenital diaphragmatic hernia-associated pulmonary hypertension. Semin Pediatr Surg. 2017;26:147–53.

    Article  Google Scholar 

  3. Kinsella JP, Ivy DD, Abman SH. Pulmonary vasodilator therapy in congenital diaphragmatic hernia: acute, late, and chronic pulmonary hypertension. Semin Perinatol. 2005;29:123–8.

    Article  Google Scholar 

  4. Kipfmueller F, Heindel K, Schroeder L, Berg C, Dewald O, Reutter H, et al. Early postnatal echocardiographic assessment of pulmonary blood flow in newborns with congenital diaphragmatic hernia. J Perinat Med. 2018;46:735–43.

    Article  CAS  Google Scholar 

  5. Patel N, Massolo AC, Paria A, Stenhouse EJ, Hunter L, Finlay E, et al. Early postnatal ventricular dysfunction is associated with disease severity in patients with congenital diaphragmatic hernia. J Pediatr. 2018;203:400–7.

    Article  Google Scholar 

  6. Patel N, Lally PA, Kipfmueller F, Massolo AC, Luco M, Van Meurs KP, et al. Ventricular dysfunction is a critical determinant of mortality in congenital diaphragmatic hernia. Am J Respir Crit Care Med. 2019;200:1522–30.

    Article  Google Scholar 

  7. Siebert JR, Haas JE, Beckwith JB. Left ventricular hypoplasia in congenital diaphragmatic hernia. J Pediatr Surg. 1984;19:567–71.

    Article  CAS  Google Scholar 

  8. Kinsella J, Steinhorn R, Mullen M, Hopper R, Keller R, Ivy D, et al. The left ventricle in congenital diaphragmatic hernia: implications for the management of pulmonary hypertension. J Pediatr. 2018;197:17–22.

    Article  Google Scholar 

  9. Putnam LR, Harting MT, Tsao K, Morini F, Yoder BA, Luco M, et al. Congenital diaphragmatic hernia defect size and infant morbidity at discharge. Pediatrics. 2016;138:e20162043.

    Article  Google Scholar 

  10. Alves L, Byrne A, Keller RL, Brook MM, Silverman H, Moon-grady AJ. Left heart structures in human neonates with congenital diaphragmatic hernia and the effect of fetal endoscopic tracheal occlusion. Fetal Diagn Ther. 2014;35:36–43.

    Article  Google Scholar 

  11. Lee W, Riggs T, Amula V, Tsimis M, Cutler N, Bronsteen R, et al. Fetal echocardiography: Z-score reference ranges for a large patient population. Ultrasound Obstet Gynecol. 2010;35:28–34.

    Article  CAS  Google Scholar 

  12. Mardy C, Blumenfeld YJ, Arunamata AA, Girsen AI, Sylvester KG, Halabi S, et al. In fetuses with congenital lung masses, decreased ventricular and atrioventricular valve dimensions are associated with lesion size and clinical outcome. Prenat Diagn. 2020;40:206–15.

    Article  CAS  Google Scholar 

  13. Vogel M, McElhinney DB, Marcus E, Morash D, Jennings RW, Tworetzky W. Significance and outcome of left heart hypoplasia in fetal congenital diaphragmatic hernia. Ultrasound Obstet Gynecol. 2010;35:310–7.

    Article  CAS  Google Scholar 

  14. Schneider C, McCrindle BW, Carvalho JS, Hornberger LK, McCarthy KP, Daubeney PEF. Development of Z-scores for fetal cardiac dimensions from echocardiography. Ultrasound Obstet Gynecol. 2005;26:599–605.

    Article  CAS  Google Scholar 

  15. Jani J, Nicolaides KH, Keller RL, Benachi A, Peralta CFA, Favre R, et al. Observed to expected lung area to head circumference ratio in the prediction of survival in fetuses with isolated diaphragmatic hernia. Ultrasound Obstet Gynecol. 2007;30:67–71.

    Article  CAS  Google Scholar 

  16. Romiti A, Viggiano M, Conforti A, Valfré L, Ravà L, Ciofi Degli Atti M, et al. Ultrasonographic assessment of mediastinal shift angle (MSA) in isolated left congenital diaphragmatic hernia for the prediction of postnatal survival. J Matern Neonatal Med. 2018;0:1–6.

    Google Scholar 

  17. Byrne FA, Keller RL, Meadows J, Miniati D, Brook MM, Silverman NH, et al. Severe left diaphragmatic hernia limits size of fetal left heart more than does right diaphragmatic hernia. Ultrasound Obstet Gynecol. 2015;46:688–94.

    Article  CAS  Google Scholar 

  18. Kailin JA, Dhillon GS, Maskatia SA, Cass DL, Shamshirsaz AA, Mehollin-ray AR, et al. Fetal left-sided cardiac structural dimensions in left-sided congenital diaphragmatic hernia–association with severity and impact on postnatal outcomes. Prenat Diagn. 2017;37:502–9.

    Article  CAS  Google Scholar 

  19. Stressig R, Fimmers R, Eising K, Gembruch U, Kohl T. Preferential streaming of the ductus venosus and inferior caval vein towards the right heart is associated with left heart underdevelopment in human fetuses with left-sided diaphragmatic hernia. Heart. 2010;96:1564–8.

    Article  Google Scholar 

  20. Duy D, Patel N, Harting MT, Lally KP, Lally PA, Buchmiller TL. Early left ventricular dysfunction and severe pulmonary hypertension predict adverse outcomes in ‘low-risk’ congenital diaphragmatic hernia. Pediatr Crit Care Med. 2020;21:637–46.

    Article  Google Scholar 

  21. Crawford DC, Wright VM, Drake DP, Allan LD. Fetal diaphragmatic hernia: the value of fetal echocardiography in the prediction of postnatal outcome. BJOG Int J Obstet Gynaecol. 1989;96:705–10.

    Article  CAS  Google Scholar 

  22. Yamoto M, Tanaka Y, Fukumoto K, Miyake H, Nakajima H, Koyama M, et al. Cardiac fetal ultrasonographic parameters for predicting outcomes of isolated left-sided congenital diaphragmatic hernia. J Pediatr Surg. 2015;50:2019–24.

    Article  Google Scholar 

  23. Mandell EW, Kinsella JP. Left ventricular dysfunction and persistent perfusion abnormalities in infants with congenital diaphragmatic hernia. J Pediatr. 2019;219:7–8.

    Article  Google Scholar 

  24. Massolo AC, Paria A, Hunter L, Finlay E, Davis CF, Patel N. Ventricular dysfunction, interdependence, and mechanical dispersion in newborn infants with congenital diaphragmatic hernia. Neonatology. 2019;116:68–75.

    Article  Google Scholar 

  25. Oluyomi-obi T, Kuret V, Puligandla P, Lodha A, Lee-robertson H, Lee K, et al. Antenatal predictors of outcome in prenatally diagnosed congenital diaphragmatic hernia (CDH). J Pediatr Surg. 2017;52:881–8.

    Article  Google Scholar 

  26. Tan J, Silverman NH, Hoffman JIE, Villegas M, Schmidt KG. Cardiac dimensions determined by cross-sectional echocardiography in the normal human fetus from 18 weeks to term. Am J Cardiol. 1992;70:1459–67.

    Article  CAS  Google Scholar 

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Acknowledgements

We are grateful to the Dr. Irma Capolupo for her assistance with the study, to Dr. Luciano Pasquini and Dr. Alessandra Toscano for their expert advice in preparation of the manuscript.

Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Author information

Author notes

  1. These authors contributed equally: Anna Claudia Massolo, Anita Romiti

Authors and Affiliations

  1. Department of Medical and Surgical Neonatology, Bambino Gesù Children’s Hospital, IRCCS, Rome, Italy

    Anna Claudia Massolo, Anita Romiti, Milena Viggiano, Chiara Vassallo, Leonardo Caforio & Pietro Bagolan

  2. Unità Operativa Complessa di Patologia Ostetrica, Dipartimento Scienze della Salute della Donna, del Bambino e di Sanità Pubblica, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy

    Chiara Vassallo

  3. Istituto di Clinica Ostetrica e Ginecologica, Università Cattolica del Sacro Cuore, Rome, Italy

    Chiara Vassallo

  4. Department of Fetal and Maternal Medicine, Queen Elizabeth University Hospital, Glasgow, UK

    Marie Anne Ledingham

  5. Department of Neonatology, Royal Hospital for Children, Glasgow, UK

    Antonio Lanzone & Neil Patel

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  1. Anna Claudia Massolo
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All authors met ICMJE authorship criteria.

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Correspondence to Anna Claudia Massolo.

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The research was conducted ethically in accordance with the World Medical Association Declaration of Helsinki.

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Massolo, A.C., Romiti, A., Viggiano, M. et al. Fetal cardiac dimensions in congenital diaphragmatic hernia: relationship with gestational age and postnatal outcomes. J Perinatol 41, 1651–1659 (2021). https://doi.org/10.1038/s41372-021-00986-y

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