CONGENITAL MISALIGNMENT OF PULMONARY VESSELS - AN UNUSUAL SYNDROME-ASSOCIATED WITH PPHN
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AbstractWe report a case of misalignment of pulmonary vessels and review the clinical features of all 13 cases reported to date. All were term infants dying from severe persistent pulmonary hypertension of the newborn. We have identified a triad of features that will alert neonatal clinicians to the possibility of this diagnosis: association with other non-lethal congenital malformations; delayed onset of presentation (especially after 12 h); and severe hypoxaemia refractory to conventional therapy. We recommend that any autopsy on newborn infants include a specific search for misalignment of pulmonary vessels to outline the pathophysiology and clinical significance of this disorder.
All Author(s) ListNG PC, LEWINDON PJ, SIU YK, TO KF, WONG W
Journal nameActa Paediatrica
Year1995
Month3
Day1
Volume Number84
Issue Number3
PublisherSCANDINAVIAN UNIVERSITY PRESS
Pages349 - 353
ISSN0803-5253
LanguagesEnglish-United Kingdom
KeywordsCONGENITAL ALVEOLAR CAPILLARY DYSPLASIA; CONGENITAL ANOMALIES; MISALIGNMENT OF PULMONARY VESSELS; PERSISTENT PULMONARY HYPERTENSION OF THE NEWBORN
Web of Science Subject CategoriesPediatrics; PEDIATRICS

Last updated on 2020-17-10 at 02:16