ECMO in CDH: Is there a role?

https://doi.org/10.1053/j.sempedsurg.2017.04.006Get rights and content

Abstract

Despite wide use and decades of experience, survival of congenital diaphragmatic hernia (CDH) patients treated with extra-corporeal membrane oxygenation (ECMO), as reported by the extra-corporeal life support organization (ELSO), remains unchanged at 50%. High-survival rates both with and without utilizing ECMO have been reported, fueling questions about the utility of ECMO support in this difficult population. This review looks at data from the Congenital Diaphragmatic Hernia Study Group and individual center reports, to evaluate the role of ECMO in CDH, focusing on defining the patients most likely to benefit, and discussing how those benefits can best be achieved. These data show that ECMO improves survival in those CDH patients who are most severely affected, but potential complications of ECMO delivery outweigh benefit in patients with less severely affected. Improved results can be expected by minimizing ECMO complications, and by improving rates of CDH repair in patients that require ECMO.

Introduction

In 1977, German et al.1 reported the first four CDH patients supported with ECMO, of which one survived. Four decades later, CDH is the most common indication for ECMO in neonates.2 Despite its wide availability and use, however, many remain unsure of the utility and benefit of this invasive support in this most difficult population. Multiple centers report excellent survival results using ECMO in 35–50% in their CDH population, but in stark contrast, other centers report very good results using ECMO sparingly, if at all. Are these experiences comparable? Several monographs specifically addressing this question of utility of ECMO in CDH have failed to derive clear conclusions.3, 4

The goal of this review is to first address then go beyond the binary question of whether ECMO improves survival in CDH, asking for which CDH patients does ECMO increase survival, how are those results best obtained, and can those results be improved? Is there an outcome difference in veno-venous (VV) versus veno-arterial (VA) ECMO in CDH? What is the optimal timing for CDH repair in the ECMO patient? Unfortunately, reporting of risk stratification data to allow direct comparison of results between series is insufficient in most reports. The task is further complicated by the fact that the ultimate survival success of ECMO in CDH is affected not only by the quality of the ECMO, but also by what comes before and after the ECMO run.

In addressing these questions, we will look to published data from the Congenital Diaphragmatic Hernia Study Group, and from individual center reports focusing on contemporary series that report high-survival rates both with, and without utilizing ECMO. We will also look at how overall treatment strategies affect CDH–ECMO outcomes, specifically the role of patient selection, ventilator support strategies, type of ECMO, and importantly, how timing of surgical repair affects CDH–ECMO outcomes.

Section snippets

History

ECMO was first used in 1977 and expanded in the 1980s to rescue neonates suffering from life-threatening hypoxemia and hypercarbia following emergent surgical repair of CDH, their courses also complicated by the ravages of aggressive ventilator support. Langham, Stolar, Newman, and many others demonstrated the life-saving potential of lung rest along with the resolution of pulmonary hypertension, which often occurred with the use of veno-arterial ECMO post-repair.4, 5, 6 In those three early

CDH spectrum of disease

In 1999, a seminal report from the CDH Study Group defined the relationship between CDH disease severity, and the survival benefit from ECMO. Compared to conventional therapy alone, ECMO support significantly improved survival for those CDH neonates with a predicted mortality greater than 80%, as defined by a logistic regression equation based on birth weight and 5-min Apgar scores.15 In contrast, when ECMO was used on patients with less severe disease, ECMO support exerted either an equivocal

Trials and series

Randomized controlled data on the role of ECMO in CDH is limited to two early ECMO studies, and the UK ECMO trial.26, 27, 28 In the UK ECMO trial, randomization to conventional ventilation versus ECMO occurred at an oxygenation index of 40. Seventeen of 17 CDH infants randomized to conventional management died, while 4 of 18 in the ECMO arm survived (0% survival versus 22% survival). One of those survivors subsequently died at 2 years of age.

In 2006, Morini et al.4 published a systematic review

References (54)

  • K.P. Lally et al.

    Standardized reporting for congenital diaphragmatic hernia—an international consensus

    J Pediatr Surg

    (2013)
  • L. Migliazza et al.

    Retrospective study of 111 cases of congenital diaphragmatic hernia treated with early high-frequency oscillatory ventilation and presurgical stabilization

    J Pediatr Surg

    (2007)
  • J.M. Zalla et al.

    Improved mortality rate for congenital diaphragmatic hernia in the modern era of management: 15 year experience in a single institution

    J Pediatr Surg

    (2015)
  • A.P. Stoffan et al.

    Does the ex utero intrapartum treatment to extracorporeal membrane oxygenation procedure change outcomes for high-risk patients with congenital diaphragmatic hernia?

    J Pediatr Surg

    (2012)
  • D.W. Kays et al.

    Outcomes in the physiologically most severe congenital diaphragmatic hernia (CDH) patients: whom should we treat?

    J Pediatr Surg

    (2015)
  • M.B. Antonoff et al.

    Protocolized management of infants with congenital diaphragmatic hernia: effect on survival

    J Pediatr Surg

    (2011)
  • R.A. Dimmitt et al.

    Venoarterial versus venovenous extracorporeal membrane oxygenation in congenital diaphragmatic hernia: the Extracorporeal Life Support Organization Registry, 1990–1999

    J Pediatr Surg

    (2001)
  • Y.S. Guner et al.

    Outcome analysis of neonates with congenital diaphragmatic hernia treated with venovenous vs venoarterial extracorporeal membrane oxygenation

    J Pediatr Surg

    (2009)
  • A. Kugelman et al.

    Venovenous versus venoarterial extracorporeal membrane oxygenation in congenital diaphragmatic hernia

    J Pediatr Surg

    (2003)
  • B. Frenckner et al.

    Neonates with congenital diaphragmatic hernia have smaller neck veins than other neonates—an alternative route for ECMO cannulation

    J Pediatr Surg

    (2002)
  • J.M. Wilson et al.

    Congenital diaphragmatic hernia—a tale of two cities: the Boston experience

    J Pediatr Surg

    (1997)
  • R. Keijzer et al.

    Congenital diaphragmatic hernia: to repair on or off extracorporeal membrane oxygenation?

    J Pediatr Surg

    (2012)
  • D.W. Kays et al.

    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)
  • C.S. Barrett et al.

    Outcomes of neonates undergoing extracorporeal membrane oxygenation support using centrifugal versus roller blood pumps

    Ann Thorac Surg

    (2012)
  • C.A. Reickert et al.

    Congenital diaphragmatic hernia survival and use of extracorporeal life support at selected level III nurseries with multimodality support

    Surgery

    (1998)
  • P. Bagolan et al.

    Impact of a current treatment protocol on outcome of high-risk congenital diaphragmatic hernia

    J Pediatr Surg

    (2004)
  • G. Casaccia et al.

    Birth weight and McGoon Index predict mortality in newborn infants with congenital diaphragmatic hernia

    J Pediatr Surg

    (2006)
  • Cited by (55)

    • Pediatric surgical interventions on ECMO

      2023, Seminars in Pediatric Surgery
    • Extracorporeal Membrane Oxygenation Then and Now; Broadening Indications and Availability

      2023, Critical Care Clinics
      Citation Excerpt :

      Survival for meconium aspiration syndrome remains more than 90%.30 Survival of congenital diaphragmatic hernia has remained relatively unchanged, hovering between 50% and 60% but with reports of higher survival rates at select centers.67,68 Efforts to improve care have attempted to standardized pre-ECMO care such as permissive hypercapnia, and targeted management of pulmonary hypertension (Table 1).69

    • Neonatal Transport

      2023, Avery's Diseases of the Newborn
    • Management of the CDH patient on ECLS

      2022, Seminars in Fetal and Neonatal Medicine
    View all citing articles on Scopus
    View full text