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


METHODS
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.


RESULTS
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.


CONCLUSIONS
We conclude that specific PAH therapy may be of benefit to patients with Sch-PAH, considering clinical, functional, and hemodynamic parameters.
Cardio-Pulmonary Department, Heart Institute, University of São Paulo Medical School, São Paulo, Brazil. Electronic address: cjcfernandes@yahoo.com.br.
Caio J C S
CJCS
Fernandes
Cardio-Pulmonary Department, Heart Institute, University of São Paulo Medical School, São Paulo, Brazil.
Bruno A
BA
Dias
Cardio-Pulmonary Department, Heart Institute, University of São Paulo Medical School, São Paulo, Brazil.
Carlos V P
CVP
Jardim
Cardio-Pulmonary Department, Heart Institute, University of São Paulo Medical School, São Paulo, Brazil.
Andre
A
Hovnanian
Cardio-Pulmonary Department, Heart Institute, University of São Paulo Medical School, São Paulo, Brazil.
Susana
S
Hoette
Cardio-Pulmonary Department, Heart Institute, University of São Paulo Medical School, São Paulo, Brazil.
Luciana K
LK
Morinaga
Cardio-Pulmonary Department, Heart Institute, University of São Paulo Medical School, São Paulo, Brazil.
Silvia
S
Souza
Cardio-Pulmonary Department, Heart Institute, University of São Paulo Medical School, São Paulo, Brazil.
Milena
M
Suesada
Cardio-Pulmonary Department, Heart Institute, University of São Paulo Medical School, São Paulo, Brazil.
Ana P
AP
Breda
Cardio-Pulmonary Department, Heart Institute, University of São Paulo Medical School, São Paulo, Brazil.
Rogério
R
Souza
D004360:Drug Therapy, Computer-Assisted; D065128:Endothelin Receptor Antagonists; D065627:Familial Primary Pulmonary Hypertension; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology* / Q000503:physiopathology; D008875:Middle Aged; D058986:Phosphodiesterase 5 Inhibitors / Q000008:administration & dosage / Q000627:therapeutic use*; D011652:Pulmonary Circulation; D012189:Retrospective Studies; D012552:Schistosomiasis / Q000150:complications*; D016896:Treatment Outcome; D014655:Vascular Resistance; D016138:Walking
D065128:Endothelin Receptor Antagonists; D058986:Phosphodiesterase 5 Inhibitors