Pulmonary hypertension: pathogenesis and management

Notable progress has been achieved in our understanding of the pathogenesis of pulmonary hypertension, in particular the role of vasodilators, vasoconstrictors and the intracellular signaling pathways, cytokines, chemokines and growth factors involved. A comprehensive history and clinical examination is mandatory in the assessment and determination of the cause of pulmonary hypertension. This should be complemented by a rationale approach to investigation. Treatment of secondary pulmonary hypertension involves targeting the underlying cause. General measures include oxygen therapy and judicious use of diuretics in patients with overt right heart failure. Newer therapies that have been developed for the treatment of idiopathic pulmonary hypertension include prostanoids, modulators of vascular remodelling such as bosentan and its analogues and PGE-5 inhibitors such as sildenafil. New therapies are likely to become available in the future as our understanding of the pathogenesis of pulmonary hypertension evolves.