Advances in therapeutic interventions for patients with pulmonary arterial hypertension

M Humbert, EMT Lau, D Montani, X Jaïs, O Sitbon… - Circulation, 2014 - Am Heart Assoc
M Humbert, EMT Lau, D Montani, X Jaïs, O Sitbon, G Simonneau
Circulation, 2014Am Heart Assoc
Pulmonary arterial hypertension (PAH) is a disease characterized by progressive
remodeling of the distal pulmonary arteries, resulting in the loss of vascular cross-sectional
area and elevated pulmonary vascular resistance (PVR). 1 Without intervention, PAH is
usually progressive, leading to right heart failure and death. Since the first reported studies
of calcium channel blockers> 30 years ago, 2, 3 we have witnessed major advances in the
therapeutic armamentarium available to treat this devastating condition. The development of …
Pulmonary arterial hypertension (PAH) is a disease characterized by progressive remodeling of the distal pulmonary arteries, resulting in the loss of vascular cross-sectional area and elevated pulmonary vascular resistance (PVR). 1 Without intervention, PAH is usually progressive, leading to right heart failure and death. Since the first reported studies of calcium channel blockers> 30 years ago, 2, 3 we have witnessed major advances in the therapeutic armamentarium available to treat this devastating condition. The development of drugs that specifically target pathways involved in disease pathogenesis has led to improvements in the quality of life and clinical outcomes in patients with PAH. At present, there are 10 drugs approved for PAH by regulatory authorities worldwide. This review will focus on recent advances in pharmacological therapy in adult PAH, including our current treatment approaches and potential future strategies.
Am Heart Assoc