Background

Diagnosis of PAH is often delayed and a diagnosis of exclusion. Lack of specific symptoms and their overlap with many other conditions pose a considerable diagnostic challenge.

Aims

This study analysed the patient journey for a global, cross-sectional cohort of PAH patients from initial symptoms to diagnosis.

Methods

A real-world survey and chart review (Adelphi PAH Disease Specific Programme (DSP?)) was used to understand the patient journey in the US, Europe (France, Germany, Italy, Spain, UK) and Japan. Data collected: March-August 2022.

Results

Patients reported first noticing their PAH symptoms at mean 54 years [SD 14], ranging from 50 [SD 12] in the UK to 56 [SD 12] in the US. Most frequently reported initial symptoms were shortness of breath (88%), fatigue (59%) and asthenia (38%). Most patients reported first seeing a primary care physician regarding their PAH symptoms (74%). Initial consultation with a specialist (pulmonologist, cardiologist, rheumatologist) was more common in Japan (44%). Most patients reported being referred to a specialist for additional testing prior to a PAH diagnosis (69%), with patients most frequently recalling undergoing an echocardiogram (86%), blood testing (67%) and RHC (63%). On average, patients reported seeing more than 2 healthcare professionals prior to PAH diagnosis, which took 10 [SD 15] months to make. 28% of patients received a misdiagnosis prior to PAH diagnosis, most commonly Chronic Obstructive Pulmonary Disease (COPD, 28%), Asthma (21%) and Anxiety (18%).

Conclusion

One quarter of the patients report initially receiving a different diagnosis than PAH. Almost all patients had an echo, but more than 35% did not recall RHC; a prolonged time to diagnosis remains common.