A prolonged time to diagnosis in pulmonary arterial hypertension (PAH) remains common, according to a poster presented at the ERS International Congress 2023 in Milan, Italy. 

Despite efforts to improve diagnostic algorithms for PAH, many patients remain undiagnosed for long periods of time. This is likely due to the nonspecific nature of many of the symptoms of PAH. Nevertheless, this scenario results in considerable disease progression before any treatment is administered, worsening clinical outcomes and prognosis. 

White and colleagues sought to analyze the patient journey to identify the typical experience of patients from the first signs of symptoms until they received a diagnosis of PAH. This was a global, cross-sectional study using real-world survey and chart review data from the Adelphi PAH Disease Specific Programme. This database contains clinical information from patients across the United States and in France, Germany, Italy, Spain, the United Kingdom, and Japan. Data was collected between March 2022 and August 2022. 

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The research team reported that patients first noticed symptoms associated with PAH at a mean age of 54 years; these were mainly dyspnea (88%), fatigue (59%), and asthenia (38%). Most often, patients presented to their primary care physician regarding these symptoms (74%), although individuals in Japan were more likely to have an initial consultation with a specialist in pulmonology, cardiology, and/or rheumatology (44%). 

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Most patients describe being referred to a specialist for additional investigations (69%), with most recalling an echocardiogram (86%), blood workup (67%), and/or right heart catheterization (63%). Overall, patients needed to see 2 healthcare professionals before receiving a PAH diagnosis, and the mean time from symptom presentation to diagnosis was 10 months. In addition, misdiagnosis was common; physicians most often mistook PAH for chronic obstructive pulmonary disease (28%), asthma (21%), and anxiety (18%). 

“One quarter of the patients report initially receiving a different diagnosis than PAH,” White and colleagues wrote. “A prolonged time to diagnosis remains common.” 


White RJ, Kwok R, Harley J, Small M, Vizza CD, Lautsch D. Diagnosing pulmonary arterial hypertension in the real world. Poster presented at: European Respiratory Society International Congress, Milan, Italy; Sept. 9-13, 2023. Poster PA1190.