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

• Published in: Therapeutic advances in respiratory disease Vol. 18; p. 17534666231218886
• Main Authors: Small, Mark, Perchenet, Loïc, Bennett, Alex, Linder, Jörg
• Format: Journal Article
• Language: English
• Published: England SAGE Publishing 01.01.2024
• Subjects: Adult; Cross-Sectional Studies; Delayed Diagnosis; Familial Primary Pulmonary Hypertension; Female; Humans; Male; Middle Aged; Pulmonary Arterial Hypertension - diagnosis; Surveys and Questionnaires; patient-reported data; diagnostic delay; patient survey; pulmonary hypertension
• ISSN: 1753-4666; 1753-4666
• DOI: 10.1177/17534666231218886

Pulmonary arterial hypertension (PAH) is a life-threatening, progressive disease often diagnosed late in its course. 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. Cross-sectional survey conducted in five European countries (EU5), Japan and the USA. 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. 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 2.0 in Japan/USA) before being diagnosed. Diagnosis was most often made by cardiologists (50.4%) or pulmonologists (49.3%). 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.