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Suspect
PAH is a devastating, rapidly progressive disease with high mortality rates.1,2 Unfortunately, the vast majority of patients are still diagnosed late in the course of disease, when they have a severe clinical impairment that predicts poor prognosis.1,3,4
Reasons for this delayed diagnosis include:
- Early in the course of PAH, even though patients have elevated pulmonary arterial pressure (PAP) and pulmonary vascular resistance (PVR), they are asymptomatic because of the stability of cardiac output5
- Diagnosis of PAH is a process of exclusion, so can require numerous tests over a period of time1
- The cause of PAH is generally unknown and the pathology is complex1
Advances in therapy and knowledge in this area provide hope for PAH patients, and proper assessment and treatment at a PAH expert centre provides them with gold-standard management of their disease.
Importance of early diagnosis of PAH
Associate Professor Eugene Kotlyar
It’s important to note that ‘unexplained breathlessness’ is not a diagnosis and should be investigated until a definitive cause is found.
By the time PAH is definitively diagnosed, ≥50% of pulmonary circulation may already be compromised.6
Early diagnosis and intervention can delay disease progression and improve patient outcomes.3 As such, it is extremely important to recognise PAH symptoms and patient types, and be aware of what to look for in the physical examination.
