PT  - JOURNAL ARTICLE
AU  - Piloto, Bruna Mamprim
AU  - Castro, Marcela Araújo
AU  - Gavilanes, Francisca
AU  - Alves, José Leonidas
AU  - Prada, Luis Felipe Lopes
AU  - Morinaga, Luciana Tamie Kato
AU  - Hoette, Susana
AU  - Jardim, Carlos
AU  - Fernandes, Caio Julio Cesar dos Santos
AU  - Souza, Rogerio
TI  - Effect of targeted therapies on survival of schistosomiasis associated pulmonary arterial hypertension
AID  - 10.1183/1393003.congress-2017.PA3524
DP  - 2017 Sep 01
TA  - European Respiratory Journal
PG  - PA3524
VI  - 50
IP  - suppl 61
4099  - http://erj.ersjournals.com/content/50/suppl_61/PA3524.short
4100  - http://erj.ersjournals.com/content/50/suppl_61/PA3524.full
SO  - Eur Respir J2017 Sep 01; 50
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.