title: PINOT NOIR: pulmonic insufficiency improvement with nitric oxide inhalational response.
abstract: BACKGROUND
  Tetralogy of Fallot (TOF) repair and pulmonary valvotomy for pulmonary stenosis (PS) lead to progressive pulmonary insufficiency (PI), right ventricular enlargement and dysfunction. This study assessed whether pulmonary regurgitant fraction measured by cardiovascular magnetic resonance (CMR) could be reduced with inhaled nitric oxide (iNO).
  
  
  METHODS
  Patients with at least moderate PI by echocardiography undergoing clinically indicated CMR were prospectively enrolled. Patients with residual hemodynamic lesions were excluded. Ventricular volume and blood flow sequences were obtained at baseline and during administration of 40 ppm iNO.
  
  
  RESULTS
  Sixteen patients (11 with repaired TOF and 5 with repaired PS) completed the protocol with adequate data for analysis. The median age [range] was 35 [19-46] years, BMI was 26 ± 5 kg/m(2) (mean ± SD), 50% were women and 75% were in NYHA class I. Right ventricular end diastolic volume index for the cohort was 157 ± 33 mL/m(2), end systolic volume index was 93 ± 20 mL/m(2) and right ventricular ejection fraction was 40 ± 6%. Baseline pulmonary regurgitant volume was 45 ± 25 mL/beat and regurgitant fraction was 35 ± 16%. During administration of iNO, regurgitant volume was reduced by an average of 6 ± 9% (p=0.01) and regurgitant fraction was reduced by an average of 5 ± 8% (p=0.02). No significant changes were observed in ventricular indices for either the left or right ventricle.
  
  
  CONCLUSIONS
  iNO was successfully administered during CMR acquisition and appears to reduce regurgitant fraction in patients with at least moderate PI suggesting a potential role for selective pulmonary vasodilator therapy in these patients.
  
  
  BACKGROUND
  ClinicalTrials.gov, NCT00543933.
authors.affiliation: Cleveland Clinic Heart and Vascular Institute, Cardiovascular Medicine, Cleveland, USA. krasusr@ccf.org.
authors.forename: Stephen A
authors.initials: SA
authors.lastname: Hart
authors.forename: Ganesh P
authors.initials: GP
authors.lastname: Devendra
authors.forename: Yuli Y
authors.initials: YY
authors.lastname: Kim
authors.forename: Scott D
authors.initials: SD
authors.lastname: Flamm
authors.forename: Vidyasagar
authors.initials: V
authors.lastname: Kalahasti
authors.forename: Janine
authors.initials: J
authors.lastname: Arruda
authors.forename: Esteban
authors.initials: E
authors.lastname: Walker
authors.forename: Thananya
authors.initials: T
authors.lastname: Boonyasirinant
authors.forename: Michael
authors.initials: M
authors.lastname: Bolen
authors.forename: Randolph
authors.initials: R
authors.lastname: Setser
authors.forename: Richard A
authors.initials: RA
authors.lastname: Krasuski
mesh_terms: D000280:Administration, Inhalation; D000328:Adult; D063126:Balloon Valvuloplasty / Q000009:adverse effects*; D006348:Cardiac Surgical Procedures / Q000009:adverse effects*; D005260:Female; D006801:Humans; D019028:Magnetic Resonance Imaging, Cine; D008297:Male; D008875:Middle Aged; D009569:Nitric Oxide / Q000008:administration & dosage*; D009820:Ohio; D011446:Prospective Studies; D011664:Pulmonary Valve / Q000187:drug effects* / Q000503:physiopathology; D011665:Pulmonary Valve Insufficiency / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D011666:Pulmonary Valve Stenosis / Q000628:therapy*; D013318:Stroke Volume / Q000187:drug effects; D013771:Tetralogy of Fallot / Q000601:surgery*; D016896:Treatment Outcome; D014665:Vasodilator Agents / Q000008:administration & dosage*; D016277:Ventricular Function, Left / Q000187:drug effects; D016278:Ventricular Function, Right / Q000187:drug effects; D055815:Young Adult
chemical_list: D014665:Vasodilator Agents; D009569:Nitric Oxide