The PAH Disability and Bothersomeness Questionnaire

"Validation of a Questionnaire Assessing Disability and Bothersomeness in Pulmonary Arterial Hypertension (PAH): The PAH Disability and Bothersomeness Questionnaire"

To develop a patient-reported questionnaire to investigate the impact of PAH (pulmonary arterial hypertension) on patients' daily lives in terms of bothersomeness and disability.

A 3-steps study protocol was initiated in 2011 to develop a patient reported outcome measure (PROM) in PAH.The first two steps have been completed (qualitative study and Delphi consensus study) leading to the development of a provisional 37-items questionnaire. A reduction of items in this provisional questionnaire and the validation of the final questionnaire are the goals of the current study.

Inclusion Criteria:

• patients with idiopathic and heritable PAH;
• patients over 18;
• male and female;
• NYHA Functional Class I, II, III & IV;
• Written consent signed
• Patients with a good understanding of the French - language
• Patient affiliated with a social security scheme

Exclusion Criteria:

• Patients under 18 years of age
• Patients with other pathologies associated with PAH (Group 1)
• Patients with other forms of pulmonary hypertension (groups 2, 3, 4 and 5)
• Non-consenting patients
• Patients who do not have a good understanding of the French language