Pulmonary hypertension is said to occur when the mean pulmonary arterial pressure exceeds 25 mmHg at rest or 30 mmHg during exercise. There are many causes but the term Pulmonary arterial hypertension (PAH) is used to describe a rare group of illnesses that share histopathological similarities in the small muscularised pulmonary arterioles leading to vascular remodelling (plexogenic pulmonary arteriopathy) and progressive elevation in the pulmonary vascular resistance. Left untreated, patients die as a consequence of right heart failure and the mortality approaches that of commonly encountered malignancies. There is no effective cure. Most treatment for PAH patients has focused on the endothelial cell vascular dysfunction known to occur in these disorders and indeed agents such as endothelin receptor antagonists, phosphodiesterase pathway V inhibitors and prostacyclin analogues have been shown to improve morbidity and delay rate of deterioration. More recently evidence has emerged that they may have a positive impact on survival. These agents have also been applied to treat patients with chronic thromboembolic pulmonary hypertension (CTEPH) and selected patients with CTEPH may also benefit from pulmonary thromboendarterectomy. For a small number of patients with PAH lung transplantation may be considered.
Brendan P Madden - Cardiothoracic Unit, St Georges Hospital, London, UK