Management of primary pulmonary hypertension

The onset of symptoms in primary pulmonary hypertension (PPH) is usually insidious with several years elapsing before the diagnosis is actually made. It is important that general physicians should be made aware of this fact and that they should have a high rate of suspicion of the subtle nature of the clinical presentation in this group of patients. Patients with a suspected diagnosis of PPH should be referred to specialised centres where early diagnosis and treatment can be initiated. We review the salient features of PPH and provide an insight into the various therapeutic options that are now available for this disease.