Chapter 13: Treatments for Pulmonary Arterial Hypertension Check Access

Pulmonary arterial hypertension is a serious and life-shortening disease that restricts the supply of oxygenated blood from the lungs to the heart as a result of increased pulmonary vascular resistance. The clinical symptoms range from breathlessness, through to drastically reduced exercise capacity and quality of life, and most seriously, premature death. The current treatment options comprising primarily prostacyclin and synthetic analogues, endothelin receptor antagonists and phosphodiesterase-5 inhibitors, have significantly improved 5 year survival rates over the past two decades and these options are reviewed. However, these agents are mainly vasodilatory in terms of mode of action and despite the advances achieved, long-term prognosis for patients remains poor. Emerging therapies are also reviewed and encouragingly include both agents that work via new vasodilatory mechanisms and also compounds that have the potential to be disease modifying by acting through anti-proliferative and anti-inflammatory mechanisms. The impact of orphan designation and orphan approved products is also briefly discussed.