Vascular abnormalities in human newborns with pulmonary hypertension

Abstract

Pulmonary vascular disease embodies all congenital or acquired pathologies that affect the pulmonary vasculature. One of them is pulmonary hypertension of the newborn (PHN), which is clinically characterized by a persistent high pulmonary vascular resistance postnatally and an abnormal vascular response. Morphologically, the vascular walls of the small pulmonary arteries become thickened, leading to increased resistance of these vessels and thus a worsening of gas exchange. PHN occurs as a primary disease or in association with abnormal lung development, for example as in congenital diaphragmatic hernia, and is a critical determinant of morbidity and mortality. Here we review the current knowledge about vascular abnormalities in PHN and discuss the vascular abnormalities in different conditions associated with pulmonary hypertension in human newborns in relation to recent findings from molecular biology.

Keywords::

• pulmonary hypertension of newborn
• pulmonary vascular development
• pulmonary vascular resistance

Acknowledgements

The authors would like to apologize to contributors in the field whose work we have not referred to as a result of space limitation.

Financial & competing interests disclosure

Ilona Sluiter is supported in part by the Sophia Foundation for Medical Research project SSWO number 521. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

No writing assistance was utilized in the production of this manuscript.