Pulmonary arterial hypertension (PAH) is a life-threatening cardiopulmonary disorder for which, not so long ago, the only effective treatment option was transplantation. Due to tremendous scientific progress over the past decade, many effective drug therapies are now available to treat this devastating condition.
- 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 are 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.
- Echocardiography and lung function testing are the most useful initial tests for suspected PAH. 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.
- Access to the best drug therapies is only available at expert PAH centres, and early referral is recommended.
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