title: Misalignment of pulmonary vessels with alveolar capillary dysplasia: association with atrioventricular septal defect and quadricuspid pulmonary valve.
abstract: Misalignment of pulmonary vessels (MPV) with alveolar capillary dysplasia (ACD) is a complex congenital vascular abnormality in newborn infants. This condition is often associated with persistent pulmonary hypertension, which leads to severe respiratory distress and finally to the death of the newborn. Here, two cases of MPV with ACD are reported that were associated with atrioventricular septal defects. Both cases showed a combination of a large atrial septal defect and a smaller subvalvular ventricular septal defect. In one newborn, the cardiac malformation was accompanied by a quadricuspid pulmonary valve. These cases emphasize the importance of considering MPV with ACD a possible cause for pulmonary hypertension in the newborn, especially when a concomitant atrioventricular septal defect could represent an alternative explanation for the pulmonary hypertension.
authors.affiliation: Institute of Pathology, University Hospital, Im Neuenheimer Feld 220/221, Heidelberg, Germany. Wilfried.Roth@med.uni-heidelberg.de
authors.forename: Wilfried
authors.initials: W
authors.lastname: Roth
authors.forename: Dietrich
authors.initials: D
authors.lastname: Bucsenez
authors.forename: Hendrik
authors.initials: H
authors.lastname: Bläker
authors.forename: Irina
authors.initials: I
authors.lastname: Berger
authors.forename: Philipp A
authors.initials: PA
authors.lastname: Schnabel
mesh_terms: D000015:Abnormalities, Multiple / Q000473:pathology*; D002196:Capillaries / Q000002:abnormalities; D017809:Fatal Outcome; D005260:Female; D005865:Gestational Age; D006343:Heart Septal Defects / Q000150:complications / Q000473:pathology*; D006344:Heart Septal Defects, Atrial / Q000150:complications / Q000473:pathology; D006345:Heart Septal Defects, Ventricular / Q000150:complications / Q000473:pathology; D006801:Humans; D007231:Infant, Newborn; D010547:Persistent Fetal Circulation Syndrome / Q000150:complications / Q000473:pathology; D011650:Pulmonary Alveoli / Q000002:abnormalities / Q000098:blood supply*; D011651:Pulmonary Artery / Q000002:abnormalities*; D011664:Pulmonary Valve / Q000002:abnormalities*; D011667:Pulmonary Veins / Q000002:abnormalities*