Neonatal and fetal therapy of congenital diaphragmatic hernia-related pulmonary hypertension

Arch Dis Child Fetal Neonatal Ed. 2022 Sep;107(5):458-466. doi: 10.1136/archdischild-2021-322617. Epub 2021 Dec 24.


Congenital diaphragmatic hernia (CDH) is a complex malformation characterised by a triad of pulmonary hypoplasia, pulmonary hypertension (PH) and cardiac ventricular dysfunction. Much of the mortality and morbidity in CDH is largely accounted for by PH, especially when persistent beyond the neonatal period and refractory to available treatment. Gentle ventilation, haemodynamic optimisation and pulmonary vasodilation constitute the foundations of neonatal treatment of CDH-related PH (CDH-PH). Moreover, early prenatal diagnosis, the ability to assess severity and the developmental nature of the condition generate the perfect rationale for fetal therapy. Shortcomings of currently available clinical therapies in combination with increased understanding of CDH pathophysiology have spurred experimental drug trials, exploring new therapeutic mechanisms to tackle CDH-PH. We herein discuss clinically available neonatal and fetal therapies specifically targeting CDH-PH and review the most promising experimental treatments and future research avenues.

Keywords: intensive care units; neonatal; neonatology.

Publication types

  • Review

MeSH terms

  • Female
  • Fetal Therapies*
  • Hernias, Diaphragmatic, Congenital* / complications
  • Hernias, Diaphragmatic, Congenital* / diagnostic imaging
  • Hernias, Diaphragmatic, Congenital* / therapy
  • Humans
  • Hypertension, Pulmonary* / etiology
  • Hypertension, Pulmonary* / therapy
  • Infant, Newborn
  • Lung
  • Pregnancy
  • Prenatal Diagnosis