PT  - JOURNAL ARTICLE
AU  - Felix R De Bie
AU  - Catherine M Avitabile
AU  - Luc Joyeux
AU  - Holly L Hedrick
AU  - Francesca M Russo
AU  - David Basurto
AU  - Jan Deprest
AU  - Natalie E Rintoul
TI  - Neonatal and fetal therapy of congenital diaphragmatic hernia-related pulmonary hypertension
AID  - 10.1136/archdischild-2021-322617
DP  - 2022 Sep 01
TA  - Archives of Disease in Childhood - Fetal and Neonatal Edition
PG  - 458--466
VI  - 107
IP  - 5
4099  - http://fn.bmj.com/content/107/5/458.short
4100  - http://fn.bmj.com/content/107/5/458.full
SO  - Arch Dis Child Fetal Neonatal Ed2022 Sep 01; 107
AB  - 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.No data are available.