title: Pulmonary edema caused by inhaled nitric oxide therapy in two patients with pulmonary hypertension associated with the CREST syndrome.
abstract: Pulmonary arterial hypertension (PAH) is commonly associated with the CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia) syndrome. Inhaled nitric oxide (iNO) is often used to assess acute vasoresponsiveness in patients with PAH, and reports of adverse reactions have been infrequent. We describe two of nine patients with PAH and CREST syndrome who had pulmonary edema develop during acute iNO testing. This complication was not encountered in the 46 patients with other forms of PAH tested with iNO. We suggest that iNO should be used with caution, if at all, to test acute vasoreactivity in patients with CREST syndrome.
authors.affiliation: Division of Pulmonary, Critical Care and Sleep Medicine, Rhode Island Hospital and Brown University School of Medicine, Providence, RI 02903, USA.
authors.forename: Ioana R
authors.initials: IR
authors.lastname: Preston
authors.forename: James R
authors.initials: JR
authors.lastname: Klinger
authors.forename: Jeanne
authors.initials: J
authors.lastname: Houtchens
authors.forename: David
authors.initials: D
authors.lastname: Nelson
authors.forename: Sangeeta
authors.initials: S
authors.lastname: Mehta
authors.forename: Nicholas S
authors.initials: NS
authors.lastname: Hill
mesh_terms: D000280:Administration, Inhalation; D000368:Aged; D017675:CREST Syndrome / Q000150:complications*; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000209:etiology; D008875:Middle Aged; D009569:Nitric Oxide / Q000008:administration & dosage / Q000009:adverse effects*; D011654:Pulmonary Edema / Q000139:chemically induced*
chemical_list: D009569:Nitric Oxide