Management of pulmonary hypertension in congenital diaphragmatic hernia: nitric oxide with prostaglandin-E1 versus nitric oxide alone

Pediatr Surg Int. 2008 Oct;24(10):1101-4. doi: 10.1007/s00383-008-2225-6.


Aim: Prostaglandin-E1 (PGE1) is used at most centers for treating pulmonary hypertension (PH) in congenital diaphragmatic hernia (CDH) because it has been regarded as effective. The aim of this study was to investigate the role of PGE1 for treating PH in CDH.

Methods: We reviewed 49 CDH cases with echocardiography-proven PH. PH was treated with PGE1 and nitric oxide (NO) and high frequency oscillatory ventilation (HFOV) from 1997 to 2001 (PG + NO; n = 19) and with NO and HFOV from 2002 to 2007 (NO; n = 30).

Results: Subject demographics, severity of PH, and presence of other anomalies were not significantly different between the two groups. In the PG + NO group, 12/19 (63.2%) survived (PG + NO-s) and 7/19 (36.8%) died (PG + NO-d). In the NO group, 21/30 (70.0%) survived (NO-s) and 9/30 (30.0%) died (NO-d). Survival rates were not significantly different. In the NO-s group, spontaneous closure of the ductus arteriosus (DA) was significantly earlier compared with the PG + NO-s group (P < 0.01; 4.0 +/- 0.9 vs. 9.5 +/- 2.2 days after birth). DA diameters were significantly larger in groups that died compared with groups that survived (P < 0.01), and PH persisted in groups that died. In the NO-s group, surgery was possible significantly earlier compared with the PG + NO-s group (P < 0.01; 3.75 +/- 0.67 vs. 6.12 +/- 0.78 days after birth). No NO-s case developed a PH crisis even though PGE1 was not used. Hospital stay was significantly shorter in the NO-s group compared with the PG + NO-s group (P < 0.05; 39.9 +/- 19 vs. 53.2 +/- 23 days).

Conclusion: Nitric oxide alone would appear to simplify the management of CDH with PH and provide better outcome.

MeSH terms

  • Alprostadil / therapeutic use*
  • Bronchodilator Agents / therapeutic use*
  • Drug Therapy, Combination
  • Ductus Arteriosus / pathology
  • Female
  • Hernias, Diaphragmatic, Congenital*
  • High-Frequency Ventilation
  • Humans
  • Hypertension, Pulmonary / mortality
  • Hypertension, Pulmonary / therapy*
  • Infant, Newborn
  • Length of Stay
  • Male
  • Nitric Oxide / therapeutic use*
  • Retrospective Studies
  • Treatment Outcome
  • Vasodilator Agents / therapeutic use*


  • Bronchodilator Agents
  • Vasodilator Agents
  • Nitric Oxide
  • Alprostadil