[10 years' experience in the use of vasodilator agents in the treatment of primary XXXX (P-PAH) (1977-1987)].

@entity1 @entity2514 @entity120 @entity594 @entity71049
We analyze the experience derived from the use of vasodilating therapy in @entity120 ( @entity120 ) at our institution in the last ten years. Important aspects such as complications, long-term hemodynamic effects and survival are particularly emphasized. We have consecutively studied forty @entity1 (29 females, 11 males) with a mean age of 21 +/- 1.6 years. The follow-up period is 50 +/- 8 months and the drugs used were: @entity2514 (HDL) and @entity594 ( @entity71049 ). On the basis of their hemodynamic response the @entity1 were divided into two groups: twenty responders (R) and twenty non-responders (NR). Both groups seem to have a different clinical and hemodynamic basal profile; most of the @entity1 in the R group had a better functional class (NYHA: I-II) than those NR (III-IV). Also, the R group had a lower basal Pp (49 vs 77 mmHg, p less than 0.05), lower Rp (17 vs 29 U/m2, p less than 0.05) and lower Rp/Rs ratio (0.59 vs 1.05, p less than 0.05). The R group has continued on long-term vasodilator therapy with NFD (n-13) and HDL (n-7) and most of them showed a significant improvement in their quality of life and still maintain a good hemodynamic response. Only minor side effects were associated with the medication. The NR group, on the other hand, has shown progressive clinical deterioration. Eight @entity1 in the NR group and three in the R group have died. The five-year probability of survival in the R group is 86% whereas it is only 45% in the NR group. We conclude that vasodilator therapy significantly improves quality of life, with minimum untoward effects, in selected @entity1 with @entity120 . The R group probably reflects a different @entity1 population or a different stage of the disease and therefore its better long-term prognosis cannot be attributable solely to the use of these drugs.
