ABSTRACT
A relatively rare but devastating disease Pulmonary arterial hypertension (PAH) is a relatively rare but potentially life-threatening disease. PAH is a particularly sinister condition that is, in most forms, likely to be diagnosed late and is associated with progressive clinical deterioration and premature death.1-3
The underlying processes that lead to the development of PAH are complex and the disease remains clinically silent until the right side of the heart begins to fail, initially only on exertion, but in later stages of the disease, at rest. Definitive diagnosis requires specialist skills. Invasive diagnostic procedures are necessary to determine the underlying aetiology and associated disease states. Due to the non-specific nature of the early symptom manifestations, diagnosis is typically not confirmed until up to 3 years from the initial symptom presentation, when disease pathophysiology is well developed.1-3
In recent years there has been increasing interest in the causes, consequences, and treatment of PAH. Pulmonary hypertension (PH) is defined haemodynamically as a mean pulmonary arterial pressure of >25 mmHg at rest or 30 mmHg with exercise.2 PAH is specifically diagnosed by excluding other causes of PH, particularly left heart disease. Historically, much attention focussed on idiopathic and familial PAH (formerly known as primary PAH).1-4 However, the contemporary view of PAH now recognizes a broader variety of aetiologies and associated conditions that may be targeted by PAH-specific therapies.1
