title: Sildenafil therapy in complex pulmonary atresia with pulmonary arterial hypertension.
abstract: BACKGROUND
  Complex pulmonary atresia (CPA) i.e. pulmonary atresia with ventricular septal defect and major aorto-pulmonary collaterals (MAPCAs) or Tetralogy of Fallot with MAPCAs frequently have a clinical course complicated by development of pulmonary arterial (PA) hypertension.
  
  
  METHODS
  A cross-sectional retrospective review of patients >16 years with CPA or Tetralogy of Fallot with MAPCAs and PA hypertension treated with sildenafil was conducted. Case notes were reviewed for baseline and follow-up (after sildenafil) characteristics.
  
  
  RESULTS
  Five patients, 4 female, median age 28 (range 18 to 47) years, were identified. All patients experienced symptomatic improvement: 2 of 4 wheelchair bound patients responded dramatically and walked 345 and 157 m respectively in 6 min following sildenafil therapy. One of the 4 with marked PA arborization abnormalities and severe ventricular dysfunction had initial symptomatic improvement. Another patient improved from walking less 100 m to climbing 2 flights of stairs. Arterial saturations improved in 2 cases from 70 and 60% to 87 and 84% respectively, whilst arterial saturations remained static in 1 case despite embolization of a classical Blalock-Taussig shunt. One patient with PA arborization/diminished PA bed was unable to tolerate sildenafil.
  
  
  CONCLUSIONS
  Sildenafil is well tolerated and leads to symptomatic improvement and better saturations in the majority of patients with CPA with PA hypertension when used in isolation or as an adjunct to percutaneous PA angioplasty.
authors.affiliation: Congenital Cardiac Centre, Southampton University Hospital, United Kingdom.
authors.forename: Zek S
authors.initials: ZS
authors.lastname: Lim
authors.forename: Joseph J
authors.initials: JJ
authors.lastname: Vettukattill
authors.forename: Anthony P
authors.initials: AP
authors.lastname: Salmon
authors.forename: Gruschen R
authors.initials: GR
authors.lastname: Veldtman
mesh_terms: D000293:Adolescent; D000328:Adult; D001028:Aortopulmonary Septal Defect / Q000150:complications / Q000188:drug therapy / Q000503:physiopathology; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D010879:Piperazines / Q000627:therapeutic use*; D018633:Pulmonary Atresia / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology; D011687:Purines / Q000627:therapeutic use; D012189:Retrospective Studies; D000068677:Sildenafil Citrate; D013450:Sulfones / Q000627:therapeutic use*; D013771:Tetralogy of Fallot / Q000150:complications / Q000188:drug therapy / Q000503:physiopathology; D055815:Young Adult
chemical_list: D010879:Piperazines; D011687:Purines; D013450:Sulfones; D000068677:Sildenafil Citrate