title: [Two-year follow-up of pulmonary arterial hypertension patients treated with sildenafil].
abstract: BACKGROUND
  The long-term effects of drugs developed for the control of pulmonary arterial hypertension (PAH) are little known, since multicenter studies usually last 12 to 16 weeks.
  
  
  OBJECTIVE
  To evaluate the two-year outcome of PAH patients receiving monotherapy with sildenafil (a phosphodiesterase-5 inhibitor), with regard to their functional capacity.
  
  
  METHODS
  Twenty four patients (ages between 8 and 54 years) with idiopathic PAH (IPAH, n = 9) or congenital heart disease-associated PAH (CHD-PAH, n = 15) were treated with sildenafil for two years, with daily oral doses ranging from 60 to 225 mg (tid). Physical capacity was assessed by the distance walked in the 6-minute walk test (DW6M) and by the degree of dyspnea at the end of the walk (Borg scale); peripheral oxygen saturation was also recorded (SpO(2)6M, pulse oximetry).
  
  
  RESULTS
  In the 18 patients who completed the two-year follow-up, there was a progressive and sustained increase in DW6M, both in the IPAH group (from 239 +/- 160 m to 471 +/- 66 m, p = 0.0076) and in the CHD-PAH group (from 361 +/- 144 m to 445 +/- 96 m, p = 0.0031), with improvement of dyspnea at the end of the walk (p<0.05 for both groups). No decrease in SpO(2)6M was observed in the groups; in patients with CHD-PAH, in particular, SpO(2)6M went from 77 +/- 20% to 79 +/- 16% (p = 0.5248). Five deaths occurred (three in the IPAH group) and one patient was lost to follow-up during the study period.
  
  
  CONCLUSIONS
  In a two-year follow-up, sildenafil proved useful in the control of the functional status of PAH patients, with significant improvement in both groups considered.
authors.affiliation: Instituto do Coração, São Paulo, SP, Brasil.
authors.forename: Sonia Meiken
authors.initials: SM
authors.lastname: Franchi
authors.forename: Alessandra Costa
authors.initials: AC
authors.lastname: Barreto
authors.forename: Cristina
authors.initials: C
authors.lastname: Cícero
authors.forename: Claudia Regina Pinheiro
authors.initials: CR
authors.lastname: Castro
authors.forename: Zilma Verçosa de Sá
authors.initials: ZV
authors.lastname: Ribeiro
authors.forename: Antonio Augusto
authors.initials: AA
authors.lastname: Lopes
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D004417:Dyspnea / Q000503:physiopathology; D005080:Exercise Test / Q000187:drug effects; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D010101:Oxygen Consumption / Q000502:physiology*; D058986:Phosphodiesterase 5 Inhibitors / Q000627:therapeutic use*; D010879:Piperazines / Q000627:therapeutic use*; D011446:Prospective Studies; D011687:Purines / Q000627:therapeutic use; D000068677:Sildenafil Citrate; D018709:Statistics, Nonparametric; D013450:Sulfones / Q000627:therapeutic use*; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D058986:Phosphodiesterase 5 Inhibitors; D010879:Piperazines; D011687:Purines; D013450:Sulfones; D000068677:Sildenafil Citrate