title: Inhaled nitric oxide and related therapies.
abstract: Children with congenital heart defects are at risk for perioperative pulmonary hypertension if they require corrective or palliative surgery in the first week of life or if they have defects associated with significant pulmonary overcirculation. In addition, children undergoing cavopulmonary connections for single ventricle lesions require low pulmonary vascular resistance for surgical success. Treatment of perioperative pulmonary hypertension with inhaled nitric oxide has become standard therapy in many centers. Related drugs that increase nitric oxide synthesis, including arginine and citrulline, have also been studied in the perioperative period. In this article, previous clinical trials of inhaled nitric oxide, intravenous arginine, and intravenous and oral citrulline in children with perioperative pulmonary hypertension or elevated pulmonary vascular resistance after a cavopulmonary connection are reviewed. In addition, recommendations are presented for each agent on the clinical use in the perioperative setting including clinical indications, assessment of clinical effect, and length of therapy.
authors.affiliation: Department of Pediatrics, Vanderbilt University School of Medicine and the Monroe Carell Jr. Children's Hospital at Vanderbilt, Nashville, TN, USA. rick.barr@Vanderbilt.Edu
authors.forename: Frederick E
authors.initials: FE
authors.lastname: Barr
authors.forename: Duncan
authors.initials: D
authors.lastname: Macrae
mesh_terms: D000280:Administration, Inhalation; D000284:Administration, Oral; D001120:Arginine / Q000008:administration & dosage; D006348:Cardiac Surgical Procedures; D002648:Child; D002675:Child, Preschool; D002956:Citrulline / Q000008:administration & dosage; D002986:Clinical Trials as Topic; D045462:Endothelium-Dependent Relaxing Factors / Q000008:administration & dosage*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000503:physiopathology; D007223:Infant; D007231:Infant, Newborn; D007275:Injections, Intravenous; D009569:Nitric Oxide / Q000008:administration & dosage*; D011183:Postoperative Complications / Q000188:drug therapy*
chemical_list: D045462:Endothelium-Dependent Relaxing Factors; D002956:Citrulline; D009569:Nitric Oxide; D001120:Arginine