Nationwide Data Show Delayed Correct Diagnosis for PAH

The long time to diagnosis is consistent with data pointing to pulmonary arterial hypertension (PAH) often being misdiagnosed as asthma, said the researchers, adding that the findings from their retrospective study provide valuable insight into where the diagnostic pathway lags.

Patients with pulmonary arterial hypertension (PAH) wait an average of over 2 years for a diagnosis, found a database analysis of patients nationwide.

The long time to diagnosis is consistent with data pointing to PAH often being misdiagnosed as asthma, said the researchers in Pulmonary Circulation, adding that the findings from their retrospective study provide valuable insight into where the diagnostic pathway lags.

“Our data suggest that there is room for improvement in how early PH [pulmonary hypertension] is considered a possibility by physicians. Efforts to increase awareness of PH among GPs [general practitioners] are ongoing and have not yet markedly reduced the diagnostic interval,” described the researchers. “Development of better tools and strategies for detecting PH, such as the recent InShape II algorithm for detecting CTEPH [chronic thromboembolic PH] following pulmonary embolism, the DETECT algorithm for PAH detection in SSc [systemic sclerosis], or enhancement of readily accessible investigative tests with artificial intelligence, are key to tackle the delay caused by sequential exclusion of more common causes of dyspnoea.”

Over 800,000 patients in the claims database and over 1 million patients in the electronic health record database had chronic unexplained dyspnea (CUD), representing 2 of the largest nationwide databases. Data from both databases showed a 0.1% PAH rate among patients with CUD. Time to diagnosis was 2.26 years, with this ranging from approximately 3 months to over 4 years.

Nearly 1 in 3 patients waited at least 10 months to receive a transthoracic echocardiogram (TTE). This finding, wrote the researchers, further bolsters the conclusion that there are delays within the PAH diagnostic pathway. Comparing their findings with a group of patients with PH, the researchers had similar observations.

“In contrast, we cannot draw any conclusions on whether the diagnostic interval in the overall PH cohort (also over 2 years) represents any delay, as this cohort includes many patients in whom PH has developed secondary to another condition (eg, left heart or lung disease),” wrote the researchers. “However, the time-to-TTE data show that there is a delay in the investigation symptoms in the PH cohort, as over a third of patients waited at least 10 months for a TTE. Altogether, these data—which do not capture the additional delay in patients reporting symptoms to their doctor—demonstrate that timely diagnosis of PAH remains a major unmet need.”

On average, patients with PAH received 3 TTEs, had 6 specialist visits, and had 2 hospitalizations before receiving their diagnosis. Similarly, patients with PH had an average of 2 TTEs, 4 specialist visits, and 2 hospitalizations before receiving a correct diagnosis.

The researchers noted that patients could have switched insurances during the study period, which could create delays in claims recordings, a limitation of the study. They also acknowledged a lack of historical data before patient enrollment in their insurance plan and highlighted that the patients were not representative of the US population. For example, uninsured patients and patients on Medicaid were not included in the claims database.

The group cited mixed results on time to a specialist visit. For example, for patients with PAH in the claims database, there was a median wait time of 1.2 months to the first cardiologist or pulmonologist visit. This wait time was markedly longer when just considering pulmonologist vs cardiologist visits: 7.1 vs 18 months, respectively.

The researchers noted small differences when they reviewed data from the EHR database, which showed shorter time-to-first pulmonologist visit, which they say is likely a result of the differences in patients included in the 2 databases, including the exclusion of uninsured and Medicaid-covered patients in the claims database.


Didden E, Lee E, Wyckmans J, Quinn D, Perchenet L. Time to diagnosis of pulmonary hypertension and diagnostic burden: a retrospective analysis of nationwide US healthcare data. Pulm Circ. Published online January 6, 2023. doi: 10.1002/pul2.12188

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