Pulmonary arterial hypertension (PAH) is a life-threatening disease of the small pulmonary arteries that progresses
to right heart failure and death if untreated. Early diagnosis and timely initiation of therapy is crucial to good
outcomes. PAH must be considered in any patient with unexplained breathlessness. Unexplained breathlessness
should be viewed just as seriously as chest pain. Definitive diagnosis requires right heart catheterisation.
Annual screening for PAH should be undertaken in high risk patient groups, such as those with systemic sclerosis
or past repair of a septal defect. Many efficacious drug therapies are available and combination therapy is now
the evidence-based standard of care.
Modern Medicine – March 2018