RT Journal Article
SR Electronic
T1 PAH-specific therapies improve survival in patients with pulmonary arterial hypertension associated with connective tissue diseases: A Russian single centre experience
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP P2370
VO 44
IS Suppl 58
A1 Alexander Volkov
A1 Natalia Yudkina
A1 Ekaterina Nikolaeva
A1 Ildar Kurmukov
YR 2014
UL http://erj.ersjournals.com/content/44/Suppl_58/P2370.abstract
AB Connective tissue diseases (CTD) is one of the frequent causes of the pulmonary arterial hypertension (PAH). Patients with PAH, associated with CTD have a poorer prognosis than other forms. We analyzed effect on survival PAH-therapy, including bosentan, sildenafil, iloprost and combined therapy.A prospective analysis of 42 patients with PAH-CTD (mean age 48(14) yrs., 35 SSc, 5 SLE and 2 MCTD) were performed. 23 patients were observed when PAH-specified therapy was not available in Russia. 70 CTD-patients without PAH were control group. Survival analyzed by Kaplan-Meier method. PAH-specific therapy was prescribed after PAH verification by RHC. Monotherapy received 25 patients (12 bosentan, 13 sildenafil) and 17 combined therapy. Pulmonary fibrosis and left heart diseases were excluded.Overall survival from first symptoms estimates was 100%, 97% and 87% at 1, 3 and 5 years in group receiving PAH-specific therapy, and was significantly better than those in a historical cohort of SSc pts 87%, 30% and 4% respectively. Survival in control group (98%, 81% and 78% respectively) was same with CTD-PAH pts. 7 pts (17%) died after a mean follow up 72(33) months.Conclusion: Overall survival rate was higher than in historical cohort, but 17% pts died. Our study underlines the importance of early start of treatment and importance of hemodynamic evaluation.