PULMONARY HYPERTENSION - HAVE WE GONE FROM A DEATH SENTENCE TO A "FORGETTABLE DISEASE" FOR MANY IN 30 YEARS? (Invited Lecture)



Robert C. Bourge, M.D., The University of Alabama at Birmingham, Birmingham, AL, USA

Pulmonary arterial hypertension is a group of diseases, probably with a genetic predisposition and possibly an environmental trigger, leading to elevated pulmonary arterial tree resistance, right heart failure, and a generally poor prognosis. Since the development of intravenous epoprostenol as the first approved therapy for pulmonary arterial hypertension, multiple agents have been approved and are being investigated. Based on the pathophysiology of the disease(s), therapies have been developed that affect the three main pathways believed responsible for arterial vasodilation: the endothelin pathway, the nitric acid pathway, and the prostaglandin pathway. These drugs act largely by stimulating receptors leading to vasodilation, blocking receptors that lead to vasoconstriction, and/or lessen vessel wall cell wall proliferation. In addition to the three more established pathways, new compounds are under development that affect the serotonin pathway that show promise as therapeutic options and work is also done on compounds that may reduce vessel wall inflammation in these diseases. These medical therapies include oral, transdermal, inhaled, and intravenous delivery options, some using novel automatic implanted devices. More recently, interventional therapies to improve vascular compliance in the pulmonary arteries are being studied. For some, the combination of these therapies may have the capability to transform the lives of our patients from that with a poor prognosis and the need for the continuous or frequently administered therapy, to a life with an almost “forgettable” disease, at least in terms of day to day activities.