Current treatment approaches to pulmonary arterial hypertension

Because of the complexities of the disease, early recognition and referral of patients with pulmonary arterial hypertension (PAH) to an expert centre is critically important. Advances in our understanding of the pathogenesis of PAH have translated into novel treatment strategies and pharmacotherapies. A supervised rehabilitation program, psychosocial support, and referral to palliative care might lead to improvements in exercise capacity and quality of life. At present the current medical treatments include prostanoids (parenteral, oral, and inhaled), endothelin antagonists, and agents that increase nitric oxide bioavailability. Although these agents all cause pulmonary vasodilation, they have been shown to improve outcome in patients who do not demonstrate an acute vasodilatory response. This suggests that they might also alter the abnormal proliferative vasculopathy that characterizes PAH. These agents have variably led to improvements in exercise capacity, symptoms, hemodynamics, and markers of right ventricular dysfunction. Adopting the principles of a goal-directed approach, most practitioners (and guidelines) advocate sequentially adding treatments until patients achieve a satisfactory clinical response. Emerging data suggest that upfront combination therapy might be superior to monotherapy. For patients who fail to achieve a satisfactory clinical response, parenteral prostanoids remain the medical treatment of choice. Early referral for evaluation of lung transplantation should be considered for patients who continue to demonstrate poor prognostic features or who require advanced medical treatments.