[Two-year follow-up of pulmonary arterial hypertension patients treated with sildenafil].
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.
Franchi
Sonia Meiken
SM
Instituto do Coração, São Paulo, SP, Brasil.
Barreto
Alessandra Costa
AC
Cícero
Cristina
C
Castro
Claudia Regina Pinheiro
CR
Ribeiro
Zilma Verçosa de Sá
ZV
Lopes
Antonio Augusto
AA
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
D058986:Phosphodiesterase 5 Inhibitors; D010879:Piperazines; D011687:Purines; D013450:Sulfones; D000068677:Sildenafil Citrate