Background

Pulmonary Arterial Hypertension (PAH) is a rare, progressive and incurable disease with often disabling symptoms.

Aim

We aimed to explore the severity of symptoms experienced by patients and the concordance of assessment between treating physicians? and patients? reported responses in a global cross-section.

Methods

Analysis of the Adelphi PAH Disease Specific Programme (DSP?); cross-sectional survey and chart review of PAH patients and their managing physicians collected between March and August 2022. Physicians reported their patients? PAH symptom experience in the month prior to survey date from available medical history, the same patients reported their symptom experience and both reports were matched. Symptoms are reported as frequency counts. The concordance between patient and physician reported symptoms was calculated using Cohen?s Kappa for inter-rater reliability.

Results

The kappa statistic score was 0.11 - 0.54 across key patient reported symptoms, indicating a slight, fair, or moderate agreement between patient?s and physician?s perspective (Table).

Conclusion

Physicians generally underreported symptoms compared to patients. This potentially has implications for the intensity of medical treatment regimens and the provision of supportive measures including physical rehabilitation and psychological counseling. 

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