The role of target therapies in schistosomiasis-associated pulmonary arterial hypertension

• Published in: Chest Vol. 141; no. 4; p. 923
• Main Authors: Fernandes, Caio J C S, Dias, Bruno A, Jardim, Carlos V P, Hovnanian, Andre, Hoette, Susana, Morinaga, Luciana K, Souza, Silvia, Suesada, Milena, Breda, Ana P, Souza, Rogério
• Format: Journal Article
• Language: English
• Published: United States 01.04.2012
• Subjects: Drug Therapy, Computer-Assisted; Endothelin Receptor Antagonists; Familial Primary Pulmonary Hypertension; Humans; Hypertension, Pulmonary - drug therapy; Hypertension, Pulmonary - etiology; Hypertension, Pulmonary - physiopathology; Middle Aged; Phosphodiesterase 5 Inhibitors - administration & dosage; Phosphodiesterase 5 Inhibitors - therapeutic use; Pulmonary Circulation; Retrospective Studies; Schistosomiasis - complications; Treatment Outcome; Vascular Resistance; Walking
• ISSN: 1931-3543
• DOI: 10.1378/chest.11-0483

Schistosomiasis-associated pulmonary arterial hypertension (Sch-PAH) may be one of the most prevalent forms of pulmonary arterial hypertension (PAH) worldwide. However, the clinical and hemodynamical response to specific PAH therapy in Sch-PAH is not known. We retrospectively analyzed the charts of all patients with Sch-PAH who initiated specific PAH treatment between June 2003 and June 2010 in a single PAH reference center in São Paulo, Brazil. Clinical and hemodynamical data were retrospectively collected and evaluated in two periods: baseline and posttreatment. The study population consisted of 12 patients with Sch-PAH. They were treated with phosphodiseterase-5 inhibitors (seven patients), endothelin receptor antagonists (four patients), or combination therapy (one patient). Mean treatment period was 34.9 ± 15.5 months. Patients with Sch-PAH presented significant improvements in terms of functional class, 6-min walk test distance (439 ± 85 to 492 ± 79 m, P = .032), cardiac index (2.66 ± 0.59 to 3.08 ± 0.68 L/min/m(2), P = .028), and indexed pulmonary vascular resistance (20.7 ± 11.6 to 15.9 ± 9 W/m(2), P = .038) with the introduction of specific PAH treatment. We conclude that specific PAH therapy may be of benefit to patients with Sch-PAH, considering clinical, functional, and hemodynamic parameters.