Pulmonary hypertension

Pulmonary arterial hypertension (PH) is an often-misdiagnosed lung disorder occurring as a primary idiopathic disease or as a complication of a large number of respiratory and cardiac diseases. It was previously thought to be a rare condition with a relentlessly progressive course and few treatment options. However, it is increasingly recognized in association with other conditions and recent advances have resulted in the development of effective therapies. This has focused attention on making an early and accurate diagnosis. Despite these recent advances, it is important to consider that it remains an alarming, incurable disease with a poor prognosis. PH describes a number of devastating diseases causing breathlessness, loss of exercise capacity, and death due to right-sided heart failure. This chapter begins by defining the condition, including the ESC and ERS guidelines, then goes on to give help with diagnosis and functional classification. Clinical features and investigations are outlined, as well as further assessment and key respiratory, cardiac, and haematological investigations. Specific targeted therapies, general treatment, and nursing care and advice are covered too.