RT Journal Article
SR Electronic
T1 Pulmonary vascular development in congenital diaphragmatic hernia
JF European Respiratory Review
JO EUROPEAN RESPIRATORY REVIEW
FD European Respiratory Society
SP 170104
DO 10.1183/16000617.0104-2017
VO 27
IS 147
A1 Daphne S. Mous
A1 Heleen M. Kool
A1 Rene Wijnen
A1 Dick Tibboel
A1 Robbert J. Rottier
YR 2018
UL http://err.ersjournals.com/content/27/147/170104.abstract
AB Congenital diaphragmatic hernia (CDH) is a rare congenital anomaly characterised by a diaphragmatic defect, persistent pulmonary hypertension (PH) and lung hypoplasia. The relative contribution of these three elements can vary considerably in individual patients. Most affected children suffer primarily from the associated PH, for which the therapeutic modalities are limited and frequently not evidence based. The vascular defects associated with PH, which is characterised by increased muscularisation of arterioles and capillaries, start to develop early in gestation. Pulmonary vascular development is integrated with the development of the airway epithelium. Although our knowledge is still incomplete, the processes involved in the growth and expansion of the vasculature are beginning to be unravelled. It is clear that early disturbances of this process lead to major pulmonary growth abnormalities, resulting in serious clinical challenges and in many cases death in the newborn. Here we provide an overview of the current molecular pathways involved in pulmonary vascular development. Moreover, we describe the abnormalities associated with CDH and the potential therapeutic approaches for this severe abnormality.Congenital pulmonary vascular diseases originate early during development, leading to clinical challenges http://ow.ly/EFno30hBhbv