The diagnostic journey of pulmonary arterial hypertension patients: results from a multinational real-world survey

Abstract

Background: Pulmonary arterial hypertension (PAH) is a life-threatening, progressive disease often diagnosed late in its course.

Objectives: To present patient-reported data that were captured within a large, multinational, point-in-time survey of PAH-treating physicians and their patients to better understand the diagnostic journey.

Design: Cross-sectional survey conducted in five European countries (EU5), Japan and the USA.

Methods: PAH-treating pulmonologists, cardiologists, rheumatologists or internists (USA only) completed a patient record form (PRF) for the next four consecutive adult PAH patients they saw; these patients filled in a patient self-completion (PSC) form on an anonymous, voluntary basis. Our report focuses on patient data; data are from PSC forms unless stated otherwise.

Results: Physician-reported PRFs and self-completed PSC forms were obtained for 1152 and 572 patients, respectively. Patients' mean (SD) age was 59.1 (14.0) years, 55.6% were female, and 57.3% had idiopathic PAH. Patient-reported data showed an average delay of 17.0 months between symptom onset and PAH diagnosis. This is longer than physicians estimated (13.8 months): this disparity may be partly due to the time taken by patients to consult a physician about their symptoms [9.6 months overall, longest in the USA (15.3 months)]. Most patients (71.6%) initially consulted primary care physicians about their symptoms and 76.4% of patients were referred to a specialist. Misdiagnoses occurred in 40.9% of patients [most frequent in the USA (51.3%), least common in Japan (27.6%)] and they saw an average of 2.9 physicians overall (3.5 in EU5 versus 2.0 in Japan/USA) before being diagnosed. Diagnosis was most often made by cardiologists (50.4%) or pulmonologists (49.3%).

Conclusion: Our data suggest that diagnostic delay in PAH results from patient- and physician-related factors, which differ across regions and include lack of awareness of PAH on both sides. Development of better screening strategies may help address this barrier to timely PAH diagnosis.

Keywords: diagnostic delay; patient survey; patient-reported data; pulmonary hypertension.

Figure 1.

Overview of study methodology. *Patient-reported outcomes data were matched against the PRFs completed by physicians for the same patient. PSC data were not available for every patient; participation was voluntary and consent was obtained. PAH, pulmonary arterial hypertension; PRFs, patient report forms; PSC, Patient self-completion.

Figure 2.

Symptoms of PAH first noticed by patients. PAH, pulmonary arterial hypertension.