title: Alveolar capillary dysplasia: diagnostic potential for cardiac catheterization.
abstract: OBJECTIVE
  Alveolar capillary dysplasia is a rare cause of persistent pulmonary hypertension of the newborn. Infants with this condition die despite maximal medical intervention including inhaled nitric oxide therapy and extracorporeal membrane oxygenation. To date, diagnosis of this lethal condition was made by open lung biopsy or during postmortem examination. We examined the possibility that distinct cardiac catheterization findings could be used in the diagnosis of this lethal disorder.
  
  
  METHODS
  We present three infants with fatal persistent pulmonary hypertension of the newborn refractory to extracorporeal membrane oxygenation and inhaled nitric oxide therapy, two with postmortem autopsy confirmation of alveolar capillary dysplasia. Each infant underwent cardiac catheterization to complete the diagnostic evaluations.
  
  
  RESULTS
  Significant right ventricular hypertension and normal pulmonary venous return were demonstrated, but a markedly diminished or absent capillary blush phase was noted in each infant. This finding is distinct from the normal capillary blush seen in infants with persistent pulmonary hypertension of the newborn of other etiologies.
  
  
  CONCLUSIONS
  Cardiac catheterization may provide a useful alternative to tissue examination in the diagnosis of alveolar capillary dysplasia.
authors.affiliation: Division of Neonatal and Developmental Medicine, Stanford University School of Medicine, Palo Alto, CA, USA.
authors.forename: S R
authors.initials: SR
authors.lastname: Hintz
authors.forename: J A
authors.initials: JA
authors.lastname: Vincent
authors.forename: P T
authors.initials: PT
authors.lastname: Pitlick
authors.forename: J R
authors.initials: JR
authors.lastname: Fineman
authors.forename: R H
authors.initials: RH
authors.lastname: Steinhorn
authors.forename: G E
authors.initials: GE
authors.lastname: Kim
authors.forename: W E
authors.initials: WE
authors.lastname: Benitz
mesh_terms: D000792:Angiography; D002196:Capillaries / Q000002:abnormalities; D006328:Cardiac Catheterization; D015199:Extracorporeal Membrane Oxygenation; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008168:Lung / Q000473:pathology; D008297:Male; D009569:Nitric Oxide / Q000627:therapeutic use; D010547:Persistent Fetal Circulation Syndrome / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D011650:Pulmonary Alveoli / Q000098:blood supply*; D011651:Pulmonary Artery / Q000000981:diagnostic imaging; D011667:Pulmonary Veins / Q000503:physiopathology; D016278:Ventricular Function, Right
chemical_list: D009569:Nitric Oxide