title: Inhaled nitric oxide for children with congenital heart disease and pulmonary hypertension.
abstract: BACKGROUND
  Endothelium-derived nitric oxide (NO) is a potent vasodilator and a major mediator of pulmonary vascular tone.
  
  
  METHODS
  Five infants underwent a trial of inhaled NO with hemodynamic monitoring in the operating room after atrioventricular canal repair. An additional 15 patients with congenital heart disease and refractory pulmonary hypertension were treated with inhaled NO for 1 day to 10 days postoperatively.
  
  
  RESULTS
  In the 5 infants with atrioventricular canal, corrective surgical intervention and conventional therapy (hyperventilation, inspired oxygen fraction of 0.80, and inotropic agents) lowered mean pulmonary artery pressure from 49.5 +/- 10.5 to 20.0 +/- 2.2 mm Hg (p < 0.001). Adding inhaled NO further decreased mean pulmonary artery pressure to 18.0 +/- 2.8 mm Hg (p = not significant). Inhaled NO had no effect on ventricular function curves (inflow occlusion) in this group. In the 15 patients with refractory postoperative pulmonary hypertension, 11 had a favorable response to inhaled NO, with a decrease in mean pulmonary artery pressure from 30.9 +/- 5.8 to 23.1 +/- 5.4 mm Hg (p < 0.01) in 8 patients with pulmonary artery catheters.
  
  
  CONCLUSIONS
  These studies demonstrate that inhaled NO has minimal beneficial effect on pulmonary artery pressure or cardiac output in infants after repair of atrioventricular canal. Inhaled NO is effective in decreasing PAP postoperatively in select patients with congenital heart disease and pulmonary hypertension refractory to conventional therapeutic modalities.
authors.affiliation: Division of Cardiovascular-Thoracic Surgery, Children's Memorial Hospital, Northwestern University Medical School, Chicago, Illinois 60614, USA.
authors.forename: R D
authors.initials: RD
authors.lastname: Curran
authors.forename: C
authors.initials: C
authors.lastname: Mavroudis
authors.forename: C L
authors.initials: CL
authors.lastname: Backer
authors.forename: M
authors.initials: M
authors.lastname: Sautel
authors.forename: V R
authors.initials: VR
authors.lastname: Zales
authors.forename: D L
authors.initials: DL
authors.lastname: Wessel
mesh_terms: D000280:Administration, Inhalation; D000293:Adolescent; D001794:Blood Pressure / Q000187:drug effects; D002302:Cardiac Output / Q000187:drug effects; D002648:Child; D002675:Child, Preschool; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications / Q000601:surgery*; D006439:Hemodynamics / Q000187:drug effects; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology; D007223:Infant; D007231:Infant, Newborn; D007430:Intraoperative Care; D008297:Male; D009569:Nitric Oxide / Q000008:administration & dosage* / Q000009:adverse effects; D011182:Postoperative Care; D011651:Pulmonary Artery / Q000503:physiopathology; D014655:Vascular Resistance / Q000187:drug effects; D014665:Vasodilator Agents / Q000008:administration & dosage* / Q000009:adverse effects
chemical_list: D014665:Vasodilator Agents; D009569:Nitric Oxide