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Ten years ago, most physicians considered pulmonary hypertension (PH) as a disease known as ‘primary pulmonary hypertension’ (PPH). The assessment and treatment of these patients was largely confined to specialist units based in and around lung transplant programmes. Indeed, apart from lung transplantation, effective treatment was limited to continuous intravenous prostacyclin delivered via a Hickman line, in itself usually used as a bridge to transplantation. Not surprisingly, therapeutic nihilism was rife, patients were left undiagnosed until the late stages of the disease, and the outlook for most was universally grim.
The association of PH with other conditions has of course long been recognised. Significant PH complicating advanced left heart disease was, and still is, a relative contraindication to heart transplantation, and PH in association with severe mitral stenosis often figured in Fellow of the Royal Australasian College of Physicians examination cases. The nocturnal oxygen trials in patients with advanced chronic obstructive pulmonary disease were effectively addressing the PH and consequent right heart failure related to chronic hypoxaemia. However apart for these historical associations, PH associated with other disorders such as liver disease, connective tissue diseases, haematological disorders, HIV infection, thyrotoxicosis and hereditary haemorrhagic telangiectasia to name a few, was under recognised. …
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