Table 7

Updated and new recommendations regarding targeted pulmonary vasodilation in the management of CDH-associated pulmonary hypertension77 82–84 155

Unchanged recommendationsStrength of consensusLevel of evidence
7.1 In the context of echocardiographic confirmation of supra-systemic pulmonary arterial hypertension in the absence of left ventricular dysfunction, a trial of inhaled nitric oxide (iNO) should be used, providing that lung recruitment is adequate. If there is no iNO response based on echocardiographic assessment or other parameters (clinical or laboratory), iNO should be stopped.4B-NR
7.2 Milrinone should be used to treat cardiac dysfunction, particularly if it is associated with pulmonary hypertension.4B-NR
7.3 The use of sildenafil may be considered in patients with refractory pulmonary hypertension (ie, unresponsive to iNO) or as an adjunct when weaning iNO.3B-NR
New recommendationStrength of consensusLevel of evidence
7.4 The use of prostacyclin (such as treprostinil and epoprostenol) may be considered as rescue therapy prior, during or after ECLS in infants with severe and refractory pulmonary hypertension.3C-LD
  • CDH, congenital diaphragmatic hernia; ECLS, extracorporeal life support; LD, limited data; NR, non-randomised.