Abstract
Summary

The history of pulmonary vascular disease has followed a remarkable course. It epitomises all that is good in medicine’s development. Early history involved description of the clinical presentation and natural history of the disease. These approaches were enhanced by the introduction of measurements such as chest radiography, ECGs and right heart catheterisation(RHC). A major advance occurred with detailed work on the structural abnormalities from histopathology. Finally, attempts to classify the different forms of pulmonary vascular disease were made.

The structural and physiological observations and advances in vascular pharmacology allowed the “modern” treatments to be introduced: prostacyclin (PGI2), bosentan, sildenafil and inhaled nitric oxide (NO). Each had a place and analogues.

We are now entering a new phase, which has benefited from lung transplant surgery giving access to patients’ lung cells for studies of mechanisms. Advances in genetics and the ability to identify the mutations that result in heritable pulmonary arterial hypertension (PAH) have pinpointed the target mechanisms for new therapies. This is not “controlling the pathophysiological abnormalities” but introducing therapies that may reverse the structural abnormalities of the disease. PAH is leading a modern revolution in medicine’s development.