Emerging drugs for pulmonary hypertension.
BACKGROUND
Pulmonary arterial hypertension (PAH) is a clinical syndrome characterized by structural narrowing of the small pulmonary arteries that often culminates in fatal right heart failure.


METHODS
PubMed was searched for PAH and treatment. Data from scientific meetings and pharmaceutical websites are also included. There are currently eight FDA approved drugs for PAH that fall into one of three classes: prostacyclins, endothelin-receptor antagonists and PDE-5 inhibitors. All have important limitations and morbidity and mortality remain high. Several new agents with similar mechanisms of action are in clinical development. Multiple novel therapeutic targets are being explored. New applications for PAH therapies, such as pulmonary hypertension due to left heart and lung disease, are also being investigated.


RESULTS
An understanding of currently available drugs and those in clinical development for pulmonary hypertension.


CONCLUSIONS
Drugs targeting the pulmonary vasculature have been an extremely active area of basic and clinical research for the past 20 years and will continue to be so for the foreseeable future. Considerable progress has been made, and yet there continues to be a great unmet medical need for developing more efficacious therapies.
Girgis
Reda E
RE
Johns Hopkins University, School of Medicine, Division of Pulmonary and Critical Care Medicine, USA. rgirgis@jhmi.edu
D016503:Drug Delivery Systems / Q000379:methods; D004359:Drug Therapy, Combination; D015507:Drugs, Investigational / Q000494:pharmacology / Q000627:therapeutic use*; D065128:Endothelin Receptor Antagonists; D017048:Health Care Costs / Q000706:statistics & numerical data; D006801:Humans; D006976:Hypertension, Pulmonary / Q000145:classification / Q000188:drug therapy* / Q000453:epidemiology / Q000473:pathology / Q000503:physiopathology; D010726:Phosphodiesterase Inhibitors / Q000008:administration & dosage / Q000494:pharmacology / Q000627:therapeutic use*; D044062:Prostaglandins I / Q000008:administration & dosage / Q000627:therapeutic use*; D011651:Pulmonary Artery / Q000187:drug effects / Q000473:pathology; D015398:Signal Transduction / Q000187:drug effects
D015507:Drugs, Investigational; D065128:Endothelin Receptor Antagonists; D010726:Phosphodiesterase Inhibitors; D044062:Prostaglandins I