Keep in mind the possibility of pulmonary arterial hypertension when evaluating patients with unexplained breathlessness. The availability of new therapies now means that early diagnosis and referral of patients with this rare group of disorders can make a substantial difference to patient outcome.
- Pulmonary arterial hypertension (PAH) comprises a group of rare diseases presenting as unexplained dyspnoea.
- Diagnosis of PAH is often delayed by more than 18 months.
- Consider the possibility of PAH in patients presenting with breathlessness.
- Detailed evaluation, including a right heart catheterisation, is needed for patients with PAH.
- Pulmonary hypertension is a common finding on transthoracic echocardiography, but it is rarely PAH.
- New treatments for PAH are available that can improve quality of life, exercise capacity and survival.