Open AccessPlacental vascular maldevelopment, intrauterine growth restriction, and pulmonary hypertension
Author(s) -
Mathias Maxwell,
Bitar Mireille,
Aldulescu Monica,
Birkett Robert,
Perez Marta,
Mestan Karen
Publication year - 2020
Publication title -
pulmonary circulation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.791
H-Index - 40
ISSN - 2045-8940
DOI - 10.1177/2045894020970056
Subject(s) - medicine , autopsy , pulmonary hypertension , maldevelopment , pulmonary hypoplasia , lung , intrauterine growth restriction , respiratory distress , pregnancy , necrotizing enterocolitis , cytotrophoblast , pathology , cardiology , fetus , placenta , surgery , anatomy , genetics , biology
A 33‐year‐old gravida 2, para 1 woman was noted to have early intrauterine growth restriction at 22 weeks gestation and subsequently developed severe pre‐eclampsia. She delivered a 460 g male neonate at 28 weeks. The infant was managed on non‐invasive ventilatory support and was gaining weight on enteral feeds for the first eight weeks of life, at which point he developed necrotizing enterocolitis. He then developed severe pulmonary hypertension that was refractory to maximal medical management. He died at 10 weeks of life due to hypoxemic respiratory and heart failure. Placental pathology revealed a constellation of findings consistent with maternal vascular malperfusion. Lung autopsy revealed muscularized and hypertrophied pulmonary arterioles consistent with severe pulmonary hypertension. Von Willebrand factor immunofluorescent staining of autopsy specimens suggest parallels in extent of endothelial injury. This case study illustrates our evolving knowledge of the fetal origins of neonatal lung diseases.