LAUSR

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.