title: Abnormal vascular tone in infants and children with lung hypoplasia: Findings from cardiac catheterization and the response to chronic therapy.
abstract: OBJECTIVE
  We describe four cases of chronic pulmonary hypertension in infants and children with chronic lung disease and pulmonary hypoplasia due to severe congenital diaphragmatic hernia (CDH) or congenital cystic adenomatoid malformation (CCAM). We report data from cardiac catheterization under various conditions: baseline respiratory support and room air, hyperoxic and inhaled nitric oxide challenge. We further report cardiac catheterization measures after chronic pulmonary vasodilator therapy with sildenafil alone or a combination of sildenafil and inhaled nitric oxide (three patients).
  
  
  METHODS
  Case series.
  
  
  METHODS
  Tertiary academic center.
  
  
  METHODS
  Infants and children ages 0-11 yrs with CDH (n = 3) or CCAM (n = 1) with evidence of chronic pulmonary hypertension by echocardiogram and cor pulmonale (n = 3).
  
  
  METHODS
  Catheterization and pulmonary vasodilator therapy.
  
  
  RESULTS
  Pulmonary vascular resistance, pulmonary arterial pressure, and changes in these measures were assessed. A 20% change in pulmonary vascular resistance was considered a clinically significant response. Ten catheterizations were performed in four patients. All patients had elevated pulmonary vascular resistance and pulmonary arterial pressures at initial catheterizations and significant vasodilation during inhaled nitric oxide.
  
  
  CONCLUSIONS
  Chronic lung disease following pulmonary hypoplasia from CDH and CCAM is associated with abnormal pulmonary vascular tone in infants and children with evidence of chronic pulmonary hypertension. Chronic pulmonary vasodilator therapy may improve pulmonary vascular function and enhance lung growth in infants and children who are treated during their period of potential for rapid lung growth.
authors.affiliation: Department of Pediatrics, University of California San Francisco, San Francisco, CA, USA.
authors.forename: Roberta L
authors.initials: RL
authors.lastname: Keller
authors.forename: Phillip
authors.initials: P
authors.lastname: Moore
authors.forename: David
authors.initials: D
authors.lastname: Teitel
authors.forename: Samuel
authors.initials: S
authors.lastname: Hawgood
authors.forename: John
authors.initials: J
authors.lastname: McQuitty
authors.forename: Jeffrey R
authors.initials: JR
authors.lastname: Fineman
mesh_terms: D006328:Cardiac Catheterization; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D015615:Cystic Adenomatoid Malformation of Lung, Congenital / Q000150:complications; D005260:Female; D016166:Free Radical Scavengers / Q000627:therapeutic use; D006548:Hernia, Diaphragmatic / Q000150:complications; D065630:Hernias, Diaphragmatic, Congenital; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000209:etiology / Q000601:surgery*; D007223:Infant; D008168:Lung / Q000002:abnormalities* / Q000098:blood supply*; D008171:Lung Diseases / Q000150:complications; D008297:Male; D009569:Nitric Oxide / Q000627:therapeutic use; D010879:Piperazines / Q000627:therapeutic use; D011687:Purines; D000068677:Sildenafil Citrate; D013450:Sulfones; D014665:Vasodilator Agents / Q000627:therapeutic use
chemical_list: D016166:Free Radical Scavengers; D010879:Piperazines; D011687:Purines; D013450:Sulfones; D014665:Vasodilator Agents; D009569:Nitric Oxide; D000068677:Sildenafil Citrate