CONGENITAL MISALIGNMENT OF PULMONARY VESSELS - AN UNUSUAL SYNDROME-ASSOCIATED WITH PPHN

We report a case of misalignment of pulmonary vessels and review the c linical features of all 13 cases reported to date. All were term infan ts dying from severe persistent pulmonary hypertension of the newborn. We have identified a triad of features that will alert neonatal clini cians to the possibility of this diagnosis: association with other non -lethal congenital malformations; delayed onset of presentation (espec ially after 12 h); and severe hypoxaemia refractory to conventional th erapy. We recommend that any autopsy on newborn infants include a spec ific search for misalignment of pulmonary vessels to outline the patho physiology and clinical significance of this disorder.