Extracorporeal membrane oxygenation in infants with congenital diaphragmatic hernia and cardiac malformations

doi:10.1016/0022-3468(94)90007-8

Journal of Pediatric Surgery

Volume 29, Issue 7, July 1994, Pages 878-881

https://doi.org/10.1016/0022-3468(94)90007-8 Get rights and content

Abstract

Since the introduction of neonatal extracorporeal membrane oxygenation (ECMO) in Canada, the authors have treated three infants with congenital diaphragmatic hernia (CDH) who had serious congenital cardiac anomalies (among 26 infants with CDH treated with ECMO). To determine the incidence of and outcome for infants with combined lesions who received ECMO, 19 years' data (April 1973 to October 1992) from the Extracorporeal Life Support Organization (ELSO) registry were reviewed. Seventeen infants with combined cardiac and diaphragmatic lesions were registered as receiving ECMO in the United States or Canada. Thus, the incidence of combined cardiac and diaphragmatic lesions was 2.5 per thousand neonates (17 of 6,295) receiving ECMO and 13 per thousand neonates (17 of 1,318) receiving ECMO for CDH. Five (29.4%) of the 17 infants survived. A congenital cardiac lesion may not be an absolute contraindication to ECMO in infants with CDH. Decisions to cannulate for ECMO should be based on the potential outcome of the underlying cardiac defect.

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Cited by (23)

Congenital heart disease associated with congenital diaphragmatic hernia: A systematic review on incidence, prenatal diagnosis, management, and outcome
2019, Journal of Pediatric Surgery
Citation Excerpt :
Six studies were from intercontinental databases (at least two continents) [17,30–34]. After excluding 19 articles because of overlapping cohorts, ninety-eight articles (n = 28,974 CDH patients) met the inclusion criteria [7,14,17,18,20,21,23,30–121]. Overall, out of 28,974 babies with CDH, 4427 (15%; range 1–58%) had CHD (Table 2).
The purpose of this study was to evaluate the impact of congenital heart disease (CHD) on infants with congenital diaphragmatic hernia (CDH).
Using a defined search strategy (PubMed, Cochrane, Embase, Web of Science MeSH headings), we searched studies reporting the incidence, management, and outcome of CDH infants born with associated CHD.
Of 6410 abstracts, 117 met criteria. Overall, out of 28,974 babies with CDH, 4427 (15%) had CHD, of which 42% were critical. CDH repair was performed in a lower proportion of infants with CHD (72%) than in those without (85%; p < 0.0001). Compared to CDH babies without CHD, those born with a cardiac lesion were more likely to have a patch repair (45% vs. 30%; p < 0.01) and less likely to undergo minimally invasive surgery (5% vs. 17%; p < 0.0001). CDH babies with CHD had a lower survival rate than those without CHD (52 vs. 73%; p < 0.001). Survival was even lower (32%) in babies with critical CHD.
CHD has a strong impact on the management and outcome of infants with CDH. The combination of CDH and CHD results in lower survival than those without CHD or an isolated cardiac defect. Further studies are needed to address some specific aspects of the management of this fragile CDH cohort.
Systematic review and meta-analysis.
Level III.
Contemporary outcomes in infants with congenital heart disease and bochdalek diaphragmatic hernia
2013, Annals of Thoracic Surgery
Citation Excerpt :
The majority of patients with congenital heart disease who were eligible for ECMO were eventually placed on ECMO support. Based in part on early data from the Extracorporeal Life Support Registry, a major structural heart anomaly is no longer considered to be a contraindication for ECMO at most institutions [23]. Instead, the decision to initiate ECMO should be based largely on the degree of respiratory acidosis on initial blood gases, parental considerations, and the expected outcome for all underlying congenital anomalies.
Fifteen percent of infants with congenital diaphragmatic hernia (CDH) are born with a coexisting cardiac anomaly. The purpose of this study was to evaluate contemporary outcomes in this patient population and to identify potential risk factors for in-hospital mortality.
Data from all CDH neonates with congenital heart disease managed at a single pediatric tertiary care referral center between 1997 and 2011 were retrospectively analyzed.
Forty (18%) of 216 CDH patients had a cardiac anomaly. This group was associated with a significant decrease in overall survival when compared with patients without cardiac anomaly (55% versus 81%; p = 0.001). There was no association between type of cardiac anomaly and mortality based on risk stratification according to the Risk Adjustment for Congenital Heart Surgery and The Society of Thoracic Surgeons–European Association for Cardiothoracic Surgery scoring systems (p = 0.86 and p = 0.87, respectively). Birth weight was similarly no different between survivors and nonsurvivors (2.8 ± 0.6 kg versus 2.8 ± 0.9 kg, respectively; p = 0.98). There was a trend toward increased extracorporeal membrane oxygenation use among nonsurvivors (p = 0.13). Infants with hemodynamic stability enabling subsequent cardiac repair were associated with lower mortality (p = 0.04). Survivors had a wide spectrum of long-term morbidity, but most had some evidence of neurodevelopmental impairment.
This large single-institution series suggests that the overall prognosis of infants with concomitant CDH and congenital heart disease can be quite variable, regardless of the type of heart anomaly. Hemodynamic instability and need for extracorporeal membrane oxygenation correlate with higher mortality. Although some long-term survivors have excellent outcomes, most suffer from chronic, long-term morbidities.
Does the ex utero intrapartum treatment to extracorporeal membrane oxygenation procedure change outcomes for high-risk patients with congenital diaphragmatic hernia?
2012, Journal of Pediatric Surgery
Citation Excerpt :
Another limitation is that our data collection stopped at the time of hospital discharge, and therefore, the effect of EXIT to ECMO in potentially decreasing the severity of morbidity in survivors was not analyzed in this current study. The combination of cardiac lesions in addition to CDH is an independent risk factor that portends worse outcomes [18,19], with some reports quoting decreased survival of 40% [20]. This was demonstrated in our current study as all 3 patients with cardiac disease died without apparent benefit of EXIT to ECMO.
In the most severe cases of congenital diaphragmatic hernia (CDH), significant barotrauma or death can occur before advanced therapies such as extracorporeal membrane oxygenation (ECMO) can be initiated. We have previously examined the use of the ex utero intrapartum treatment (EXIT) to ECMO procedure (EXIT with placement on ECMO) in high-risk infants and reported a survival advantage. We report our experience with EXIT to ECMO in a more recent cohort of our patients with most severe CDH.
Every patient with less than 15% predicted lung volume during January 2005 to December 2010 was included. We obtained data on prenatal imaging, size and location of the defect, and survival.
Seventeen high-risk infants were identified. All 17 (100%) received ECMO and required a patch. Six children were delivered by EXIT to ECMO, and only 2 (33%) survived. An additional patient was delivered by EXIT to intubation with ECMO on standby and died. Of the 10 children who did not receive EXIT, 5 (50%) survived.
No clear survival benefit with the use of the EXIT to ECMO procedure was demonstrated in this updated report of our high-risk CDH population. The general application of EXIT to ECMO for CDH is not supported by our results.
Shock in the Surgical Neonate
2012, Hemodynamics and Cardiology: Neonatology Questions and Controversies Expert Consult - Online and Print
Shock in the surgical neonate
2012, Hemodynamics and Cardiology
Shock in the Surgical Neonate
2008, Hemodynamics and Cardiology: Neonatology Questions and Controversies

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Extracorporeal membrane oxygenation in infants with congenital diaphragmatic hernia and cardiac malformations

Abstract

J Pediatr Surg

J Pediatr

J Pediatr Surg

J Pediatr Surg

J Pediatr

J Pediatr Surg

Neonatal diaphragmatic hernia: An improving outlook with extracorporeal membrane oxygenation

Arch Surg

Neonatal congenital diaphragmatic hernia and extracorporeal membrane oxygenation

Can Med Assoc J

Natal history of congenital diaphragmatic hernia: Implications for management

Pediatr Surg Int