Table 6

Updated recommendations regarding the role of prostaglandin E1 (PGE1) in the medical management of pulmonary hypertension associated with CDH78 79

Updated recommendationsStrength of consensusLevel of evidence
6.1 PGE1 infusions should be used:

  1. If pulmonary or systemic blood flow is dependent on patency of the ductus arteriosus.

  2. In the presence of a concomitant anatomical cardiac lesion.

4B-NR
6.2 PGE1 infusions may be considered:

  1. In the presence of supra-systemic right ventricular pressures.

  2. In the presence of right ventricular failure.

  3. If right-to-left ductal shunting exceeds left-to-right shunting.

4C-LD
6.3 PGE1 should be considered to maintain ductal patency in CDH if there is left ventricular dysfunction or functional aortic atresia in the context of systemic right ventricular or pulmonary artery pressures.4C-EO

  • CDH, congenital diaphragmatic hernia; EO, expert opinion; LD, limited data; NR, non-randomised.