Pulmonary arterial hypertension: therapeutic algorithm.

The numerous controlled clinical trials performed recently in pulmonary arterial hypertension (PAH) can allow us to abandon a clinical-based treatment strategy and adopt an evidence-based therapy. The treatment algorithm is restricted to patients in NYHA class III or IV. The different treatments have been evaluated mainly in sporadic, idiopathic PAH and in PAH associated with scleroderma or to anorexigen use. Extrapolation of these recommendations to other PAH subgroups should be done with caution. High doses of calcium channel blockers are indicated only in the minority of patients who are responders to acute vasoreactivity testing. Patients in NYHA class III should be considered candidates for treatment with either an endothelin receptor antagonist, a prostanoid or a type 5 phosphodiesterase inhibitor. Continuous intravenous administration of epoprostenol is proposed as rescue treatment in NYHA class IV patients. Combination therapy can be attempted in selected cases. Balloon atrial septostomy and/or lung transplantation are indicated for refractory patients or when medical treatments are unavailable.