RT Journal Article
SR Electronic
T1 Effect of targeted therapies on survival of schistosomiasis associated pulmonary arterial hypertension
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP PA3524
DO 10.1183/1393003.congress-2017.PA3524
VO 50
IS suppl 61
A1 Piloto, Bruna Mamprim
A1 Castro, Marcela Araújo
A1 Gavilanes, Francisca
A1 Alves, José Leonidas
A1 Prada, Luis Felipe Lopes
A1 Morinaga, Luciana Tamie Kato
A1 Hoette, Susana
A1 Jardim, Carlos
A1 Fernandes, Caio Julio Cesar dos Santos
A1 Souza, Rogerio
YR 2017
UL http://erj.ersjournals.com/content/50/suppl_61/PA3524.abstract
AB Schistosomiasis associated pulmonary arterial hypertension (Sch-PAH) is one of the most prevalent forms of pulmonary hypertension, mainly in developing countries. Although Sch-PAH seems to have a more benign clinical course than idiopathic pulmonary arterial hypertension, the 3-year mortality rate is certainly non negligible. Case series demonstrated that targeted therapies lead to significant improvement in hemodynamics but their effect on survival is still not known. The aim of this study was to compare the survival of newly diagnosed Sch-PAH patients treated with targeted therapies against a historical group of untreated patients.We retrospectively analyzed charts of all 46 consecutive patients with Sch-PAH referred to our center, from January 2008 to October 2016, comparing demographic and hemodynamic characteristics between treated and untreated patients and evaluating the 36-month survival.Results: Sch-PAH treated and untreated patients had similar age, hemodynamic impairment, clinical status and exercise capacity at the time of diagnosis. Survival rates at 36 months were 58% for untreated and 95% for treated Sch-PAH patients (p=0.005) (figure 1).Conclusion: The use of targeted therapies in Sch-PAH is associated with a significant higher survival at 36 months, reinforcing that those patients should be managed similarly to other forms of pulmonary arterial hypertension.