Since then, there has been a steady exploration of the molecular mechanisms underlying PAH, which has led to development of effective targeted therapeutic options such as endothelin receptor antagonists and prostacyclin derivatives. In Professor Rubin’s view, these options represent a major success, but he hoped that there would be further progress. Many patients may not respond to these therapies, and it remains a serious and life-threatening disease.
The characterisation of PAH into its clinical, pathobiological and epidemiological components has provided the opportunity to intervene therapeutically and to delay disease progression. Three