J Korean Med Sci.  2024 Dec;39(48):e300. 10.3346/jkms.2024.39.e300.

Enhancing the Survival of Congenital Diaphragmatic Hernia: Quality Improvement Initiative With a Multidisciplinary Extracorporeal Membrane Oxygenation Team Approach

• Lee BS
• Jung E
• Kim H
• Kim SH
• Jeong J
• Lee HN
• Kwon H
• Namgoong JM
• Kim DY

• KMID: 2562467
• DOI: http://doi.org/10.3346/jkms.2024.39.e300

Abstract

Background
Extracorporeal membrane oxygenation (ECMO) is the only treatment option that can stabilize patients with congenital diaphragmatic hernia (CDH) with severe pulmonary hypertension. This study assessed the effects of a multidisciplinary ECMO team approach (META) as part of a quality improvement initiative aimed at enhancing the survival rates of neonates with CDH.
Methods
The medical records of infants with CDH treated at a tertiary center were retrospectively reviewed. Patients were categorized into two groups based on META implementation. The META group (P2) were given key interventions, including on-site ECMO management within the neonatal intensive care unit (NICU), use of venoarterial modality, ECMO indication as a priority even before the use of inhaled nitric oxide, and preplanned surgery following ECMO discontinuation. These approaches were compared with standard protocols in the pre-META group (P1) to assess their effects on clinical outcomes, particularly in-hospital mortality.
Results
Over a 16-year period, 322 patients were included. P2 had a significantly higher incidence of non-isolated CDH and higher rate of cesarean section compared with P1. Moreover, P2 had delayed time to surgical repair (9.4 ± 8.0 days) compared with P1 (6.7 ± 7.3 days) (P = 0.004). The overall survival rate at NICU discharge was 72.7%, with a significant improvement from P1 (66.3%, 132/199) to P2 (82.9%, 102/123) (P = 0.001). Among the 68 patients who received ECMO, P2 had significantly lower baseline oxygenation index and serum lactate levels before ECMO cannulation than P1. The survival rate of patients who received ECMO also remarkably improved from P1 (21.1%, 8/38) to P2 (56.7%, 17/30). Subgroups who could be weaned from ECMO before 2 weeks after cannulation showed the best survival rate.
Conclusion
META significantly improved the survival rate of newborn infants with CDH. Further interventions, including prenatal intervention and novel ECMO strategies, may help improve the clinical outcomes and quality of life.

Keyword

Congenital Diaphragmatic Hernia; Extracorporeal Membrane Oxygenation; Survival Rate; Quality Improvement

Figure

• 

  Fig. 1 Changes in survival rates of congenital diaphragmatic hernia during the ECMO era. The numbers indicate the annual survival rate in period 1 (red bars) and period 2 (blue bars).ECMO = extracorporeal membrane oxygenation.

• 

  Fig. 2 Kaplan-Meier survival curves for congenital diaphragmatic hernia comparing period 1 and period 2.

• 

  Fig. 3 Mortality rate and duration of ECMO run. The numbers within and on top of each bar represent the number of cases and mortality rate, respectively.ECMO = extracorporeal membrane oxygenation.

Reference

1. Pober BR. Overview of epidemiology, genetics, birth defects, and chromosome abnormalities associated with CDH. Am J Med Genet C Semin Med Genet. 2007; 145C(2):158–171. PMID: 17436298.

2. Smithers CJ, Zalieckas JM, Rice-Townsend SE, Kamran A, Zurakowski D, Buchmiller TL. The timing of congenital diaphragmatic hernia repair on extracorporeal membrane oxygenation impacts surgical bleeding risk. J Pediatr Surg. 2023; 58(9):1656–1662. PMID: 36709093.

3. Gupta VS, Harting MT, Lally PA, Miller CC, Hirschl RB, Davis CF, et al. Mortality in congenital diaphragmatic hernia: a multicenter registry study of over 5000 patients over 25 years. Ann Surg. 2023; 277(3):520–527. PMID: 34334632.

4. Morini F, Goldman A, Pierro A. Extracorporeal membrane oxygenation in infants with congenital diaphragmatic hernia: a systematic review of the evidence. Eur J Pediatr Surg. 2006; 16(6):385–391. PMID: 17211783.

5. Mugford M, Elbourne D, Field D. Extracorporeal membrane oxygenation for severe respiratory failure in newborn infants. Cochrane Database Syst Rev. 2008; (3):CD001340. PMID: 18646070.

6. Guner YS, Harting MT, Jancelewicz T, Yu PT, Di Nardo M, Nguyen DV. Variation across centers in standardized mortality ratios for congenital diaphragmatic hernia receiving extracorporeal life support. J Pediatr Surg. 2022; 57(11):606–613. PMID: 35193755.

7. Jeon S, Jeong MH, Jeong SH, Park SJ, Lee N, Bae MH, et al. Perinatal prognostic factors for congenital diaphragmatic hernia: a Korean single-center study. Neonatal Med. 2022; 29(2):76–83.

8. Hwang JH, Kim CY, Park HW, Namgoong JM, Kim DY, Kim SC, et al. Trends in treatment outcome and critical predictors of mortality for congenital diaphragmatic hernia in a single center. Perinatology. 2018; 29(2):72–77.

9. Ahn JH, Jung YH, Shin SH, Kim HY, Kim EK, Kim HS. Respiratory severity score as a predictive factor for the mortality of congenital diaphragmatic hernia. Neonatal Med. 2018; 25(3):102–108.

10. Kim BE, Ha EJ, Kim YA, Kim S, Park JJ, Yun TJ, et al. Four cases of extracorporeal membrane oxygenation for congenital diaphragmatic hernia. J Korean Soc Neonatol. 2009; 16(1):64–70.

11. Jeong J, Lee BS, Cha T, Jung E, Kim EA, Kim KS, et al. Prenatal prognostic factors for isolated right congenital diaphragmatic hernia: a single center’s experience. BMC Pediatr. 2021; 21(1):460. PMID: 34666738.

12. Kim E, Sodirzhon-Ugli NY, Kim DW, Lee KS, Lim Y, Kim MC, et al. Prediction of 6-month mortality using pre-extracorporeal membrane oxygenation lactate in patients with acute coronary syndrome undergoing veno-arterial-extracorporeal membrane oxygenation. J Chest Surg. 2022; 55(2):143–150. PMID: 35232896.

13. Mungan İ, Kazancı D, Bektaş Ş, Ademoglu D, Turan S. Does lactate clearance prognosticates outcomes in ECMO therapy: a retrospective observational study. BMC Anesthesiol. 2018; 18(1):152. PMID: 30355289.

14. Guner YS, Nguyen DV, Zhang L, Chen Y, Harting MT, Rycus P, et al. Development and validation of extracorporeal membrane oxygenation mortality-risk models for congenital diaphragmatic Hernia. ASAIO J. 2018; 64(6):785–794. PMID: 29117038.

15. Partridge EA, Peranteau WH, Rintoul NE, Herkert LM, Flake AW, Adzick NS, et al. Timing of repair of congenital diaphragmatic hernia in patients supported by extracorporeal membrane oxygenation (ECMO). J Pediatr Surg. 2015; 50(2):260–262. PMID: 25638614.

16. Robertson JO, Criss CN, Hsieh LB, Matsuko N, Gish JS, Mon RA, et al. Comparison of early versus delayed strategies for repair of congenital diaphragmatic hernia on extracorporeal membrane oxygenation. J Pediatr Surg. 2018; 53(4):629–634. PMID: 29173775.

17. Bryner BS, West BT, Hirschl RB, Drongowski RA, Lally KP, Lally P, et al. Congenital diaphragmatic hernia requiring extracorporeal membrane oxygenation: does timing of repair matter? J Pediatr Surg. 2009; 44(6):1165–1171. PMID: 19524734.

18. Delaplain PT, Harting MT, Jancelewicz T, Zhang L, Yu PT, Di Nardo M, et al. Potential survival benefit with repair of congenital diaphragmatic hernia (CDH) after extracorporeal membrane oxygenation (ECMO) in select patients: Study by ELSO CDH Interest Group. J Pediatr Surg. 2019; 54(6):1132–1137. PMID: 30898399.

19. Dassinger MS, Copeland DR, Gossett J, Little DC, Jackson RJ, Smith SD, et al. Early repair of congenital diaphragmatic hernia on extracorporeal membrane oxygenation. J Pediatr Surg. 2010; 45(4):693–697. PMID: 20385272.

20. Fallon SC, Cass DL, Olutoye OO, Zamora IJ, Lazar DA, Larimer EL, et al. Repair of congenital diaphragmatic hernias on extracorporeal membrane oxygenation (ECMO): does early repair improve patient survival? J Pediatr Surg. 2013; 48(6):1172–1176. PMID: 23845603.

21. Glenn IC, Abdulhai S, Lally PA, Schlager A. Congenital Diaphragmatic Hernia Study Group. Early CDH repair on ECMO: Improved survival but no decrease in ECMO duration (A CDH Study Group Investigation). J Pediatr Surg. 2019; 54(10):2038–2043. PMID: 30898400.

22. Steen EH, Lee TC, Vogel AM, Fallon SC, Fernandes CJ, Style CC, et al. Congenital diaphragmatic hernia repair in patients on extracorporeal membrane oxygenation: how early can we repair? J Pediatr Surg. 2019; 54(1):50–54. PMID: 30482539.

23. The Neonatal Inhaled Nitric Oxide Study Group (NINOS). Inhaled nitric oxide and hypoxic respiratory failure in infants with congenital diaphragmatic hernia. Pediatrics. 1997; 99(6):838–845. PMID: 9190553.

24. Putnam LR, Tsao K, Morini F, Lally PA, Miller CC, Lally KP, et al. Evaluation of variability in inhaled nitric oxide use and pulmonary hypertension in patients with congenital diaphragmatic hernia. JAMA Pediatr. 2016; 170(12):1188–1194. PMID: 27723858.

25. Noh CY, Chock VY, Bhombal S, Danzer E, Patel N, Dahlen A, et al. Early nitric oxide is not associated with improved outcomes in congenital diaphragmatic hernia. Pediatr Res. 2023; 93(7):1899–1906. PMID: 36725908.

26. Mohseni-Bod H, Bohn D. Pulmonary hypertension in congenital diaphragmatic hernia. Semin Pediatr Surg. 2007; 16(2):126–133. PMID: 17462565.

27. Lakshminrusimha S, Keszler M. Persistent Pulmonary hypertension of the newborn. Neoreviews. 2015; 16(12):e680–e692. PMID: 26783388.

28. Lally KP, Lally PA, Langham MR, Hirschl R, Moya FR, Tibboel D, et al. Surfactant does not improve survival rate in preterm infants with congenital diaphragmatic hernia. J Pediatr Surg. 2004; 39(6):829–833. PMID: 15185206.

29. Sevilmis YD, Olutoye OO 2nd, Peiffer S, Mehl SC, Belfort MA, Rhee CJ, et al. Surfactant therapy in congenital diaphragmatic hernia and fetoscopic endoluminal tracheal occlusion. J Surg Res. 2024; 296:239–248. PMID: 38295711.

30. Van Meurs K. Congenital Diaphragmatic Hernia Study Group. Is surfactant therapy beneficial in the treatment of the term newborn infant with congenital diaphragmatic hernia? J Pediatr. 2004; 145(3):312–316. PMID: 15343181.

31. Boucherat O, Benachi A, Chailley-Heu B, Franco-Montoya ML, Elie C, Martinovic J, et al. Surfactant maturation is not delayed in human fetuses with diaphragmatic hernia. PLoS Med. 2007; 4(7):e237. PMID: 17676984.

32. Guner YS, Delaplain PT, Zhang L, Di Nardo M, Brogan TV, Chen Y, et al. Trends in mortality and risk characteristics of congenital diaphragmatic hernia treated with extracorporeal membrane oxygenation. ASAIO J. 2019; 65(5):509–515. PMID: 29863628.

33. Guner YS, Harting MT, Fairbairn K, Delaplain PT, Zhang L, Chen Y, et al. Outcomes of infants with congenital diaphragmatic hernia treated with venovenous versus venoarterial extracorporeal membrane oxygenation: a propensity score approach. J Pediatr Surg. 2018; 53(11):2092–2099. PMID: 30318280.

34. Kugelman A, Gangitano E, Pincros J, Tantivit P, Taschuk R, Durand M. Venovenous versus venoarterial extracorporeal membrane oxygenation in congenital diaphragmatic hernia. J Pediatr Surg. 2003; 38(8):1131–1136. PMID: 12891480.

35. Fukuda S, Aoyama M, Yamada Y, Saitoh N, Honjoh T, Hasegawa T, et al. Comparison of venoarterial versus venovenous access in the cerebral circulation of newborns undergoing extracorporeal membrane oxygenation. Pediatr Surg Int. 1999; 15(2):78–84. PMID: 10079335.

36. Kays DW, Talbert JL, Islam S, Larson SD, Taylor JA, Perkins J. Improved survival in left liver-up congenital diaphragmatic hernia by early repair before extracorporeal membrane oxygenation: optimization of patient selection by multivariate risk modeling. J Am Coll Surg. 2016; 222(4):459–470. PMID: 27016974.

37. Seetharamaiah R, Younger JG, Bartlett RH, Hirschl RB. Congenital Diaphragmatic Hernia Study Group. Factors associated with survival in infants with congenital diaphragmatic hernia requiring extracorporeal membrane oxygenation: a report from the Congenital Diaphragmatic Hernia Study Group. J Pediatr Surg. 2009; 44(7):1315–1321. PMID: 19573654.

38. Hoffman SB, Massaro AN, Gingalewski C, Short BL. Survival in congenital diaphragmatic hernia: use of predictive equations in the ECMO population. Neonatology. 2011; 99(4):258–265. PMID: 21088434.