Diagnostic Delays and Quality of Life in Japanese Patients with Pulmonary Hypertension: A Nationwide Survey

Abstract

Introduction: Pulmonary hypertension (PH) is a rare and severe disorder that significantly affects patients' lives. However, a comprehensive picture of the diagnosis and treatment of this condition in Japan remains unclear. This study aimed to elucidate these aspects by conducting a nationwide survey targeting patients with PH and treating physicians.

Methods: A cross-sectional survey was conducted among 160 patients with PH (119 with pulmonary arterial hypertension [PAH] and 41 with chronic thromboembolic pulmonary hypertension [CTEPH]), of whom 121 were female (75.6%), and 211 physicians across Japan. The questionnaires assessed patients' diagnostic journey, employment status, communication with physicians regarding treatment goals, health-related quality of life (HRQoL), and medication adherence.

Results: Patients visited a mean of 2.3 medical facilities before receiving a PH diagnosis (PAH patients: 2.2 visits; CTEPH patients: 2.3 visits), with a mean time from symptom onset to diagnosis of 18.0 months (PAH: 20.2 months; CTEPH: 12.2 months). Employment and school attendance rates declined from 68.8% before diagnosis to 44.4% immediately after diagnosis, and further to 36.9% at the time of the survey. Discrepancies in communication about treatment goals were observed between patients and physicians, particularly in patients with CTEPH (82.9% of patients reported such discussions vs. 41.2% of treating physicians). Median HRQoL scores, as assessed by the emPHasis-10 questionnaire, indicated impairment (PAH: 21.5; CTEPH: 18.0), which worsened with increasing disease severity.

Conclusion: This nationwide study provides a comprehensive overview of the challenges faced by patients with PH in Japan. The findings suggest the essential need for earlier diagnosis, support for employment and education among patients, and improved patient-physician communication to reduce the burden of PH and enhance patient outcomes. Graphical abstract avaliable for this article.

Keywords: Medication adherence; Patient journey; Patient-physician communication; Pulmonary hypertension; Quality of life.

Fig. 1

Flow diagram showing the recruitment of patients and physicians. CTEPH Chronic thromboembolic pulmonary hypertension, PAH pulmonary arterial hypertension

Fig. 2

Changes in work and school status in patients with PH. Change in work or school status for patients with PH from pre-diagnosis, immediately after diagnosis, to current situation (% of the total number of patients) is shown for patients with PH (a) and those stratified according to WHO-FC I and II (b-1) and III and IV (b-2). PH Pulmonary hypertension, WHO-FC World Health Organization functional class

Fig. 3

Communication regarding treatment goals between patients and physicians. The question regarding the consultation of patient’s goals was: “Are you able to consult with your primary physician for pulmonary arterial hypertension regarding the goals you want to achieve through treatment, such as work, school, travel, or sports?”. The question regarding physician’s experience of receiving consultation regarding goals was: “Have you ever received a consultation regarding what the patient wants to achieve through treatment, such as return to work, school, travel, or sports?”. Vertical axis shows the percentage and number of patients or physicians who answered yes to each posed question. CTEPH Chronic thromboembolic pulmonary hypertension, PAH pulmonary arterial hypertension