title: Prospective diagnosis of alveolar capillary dysplasia in infants with congenital heart disease.
abstract: Alveolar capillary dysplasia (ACD) is a lethal pulmonary disorder found in newborns that is characterized by severe pulmonary hypertension and hypoxemia. We report on the clinical behavior of this disorder in a series of patients and its association with congenital heart disease, especially left heart obstructive disease; we also report a prospective diagnosis of ACD by lung biopsy in a newborn with congenital heart disease, which prevented futile and prolonged medical intervention.
authors.affiliation: Department of Pediatrics, Case Western Reserve University School of Medicine, Cleveland, Ohio, USA. jrl2@po.cwru.edu
authors.forename: J R
authors.initials: JR
authors.lastname: Lane
authors.forename: E
authors.initials: E
authors.lastname: Siwik
authors.forename: T
authors.initials: T
authors.lastname: Preminger
authors.forename: E
authors.initials: E
authors.lastname: Stork
authors.forename: M
authors.initials: M
authors.lastname: Spector
mesh_terms: D001165:Arteriovenous Malformations / Q000175:diagnosis* / Q000473:pathology; D002196:Capillaries / Q000473:pathology; D017809:Fatal Outcome; D005260:Female; D006330:Heart Defects, Congenital / Q000175:diagnosis* / Q000473:pathology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000473:pathology; D000860:Hypoxia / Q000175:diagnosis / Q000473:pathology; D007231:Infant, Newborn; D008297:Male; D011379:Prognosis; D011446:Prospective Studies; D011650:Pulmonary Alveoli / Q000098:blood supply* / Q000473:pathology; D012127:Respiratory Distress Syndrome, Newborn / Q000175:diagnosis / Q000473:pathology