Abstract

Optimisation of respiratory support of infants with congenital diaphragmatic hernia (CDH) is critical. Infants with CDH often have severe lung hypoplasia and abnormal development of their pulmonary vasculature, leading to ventilation perfusion mismatch. It is vital that lung protective ventilation strategies are employed during both initial stabilisation and post-surgical repair to avoid ventilator induced lung damage and oxygen toxicity to prevent further impairment to an already diminished gas-exchanging environment. There is a lack of robust evidence for the routine use of surfactant therapy during initial resuscitation of infants with CDH and thus administration cannot be recommended outside clinical trials. Additionally, inhaled nitric oxide has been shown to have no benefit in reducing the mortality rates of infants with CDH. Other therapeutic agents which beneficially act on pulmonary hypertension are currently being assessed in infants with CDH in randomised multicentre trials. The role of novel ventilatory modalities such as closed loop automated oxygen control, liquid ventilation and heliox therapy may offer promise for infants with CDH, but the benefits need to be determined in appropriately designed clinical trials.

Highlights

  • The developmental disruption to the lungs and pulmonary vasculature of newborn infants with congenital diaphragmatic hernia (CDH) poses challenges during adaptation to postnatal life

  • Lung volume recruitment strategies utilised by high frequency oscillatory ventilation (HFOV) were not found to be superior to conventional mechanical ventilation (CMV) in reducing the combined outcome of death or bronchopulmonary dysplasia (BPD) [odds ratio (OR) 0.62, p = 0.31]

  • Given the underlying lung pathology in CDH is that of pulmonary hypoplasia this likely explains the inferiority of HFOV compared to CMV

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Summary

Introduction

The developmental disruption to the lungs and pulmonary vasculature of newborn infants with congenital diaphragmatic hernia (CDH) poses challenges during adaptation to postnatal life. The function of the lungs in providing essential gas exchange of oxygen and carbon dioxide at the alveolar-capillary membrane can be greatly diminished in infants presenting with this congenital abnormality [1]. This review aims to provide clinicians with an outline of the recent evidence based ventilatory options available from birth and the initial stabilisation, through to surgery and post-operative management. It will provide an insight into future developments

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