Quality of Life Questionnaire in Pulmonary Hypertension EMPHASIS-10: Brazilian Adaptation and Validation (EMPHASIS-10-BR)

LOGISTICS AND STUDY PROCEDURES The use of the EmPHasis-10 questionnaire was authorized by the Pulmonary Hypertension Association - United Kingdom (PHA-UK), the organization holding its copyright, and the translation process will be conducted in accordance with the guidelines provided by PHA-UK.

The translation process will initially consist of two independent translations into Brazilian Portuguese performed by two independent translators. Subsequently, the questionnaire will be back-translated into English by a third independent translator. All translations will be reviewed by a committee composed of three pulmonologists specialized in pulmonary circulation, members of the research team, and fluent in both languages.

A review report of the back-translation will be submitted to PHA-UK, which may provide suggestions that will be forwarded to the investigators for further adjustments, if necessary. After the final version is established, the questionnaire will be pilot-tested in five patients. After completing the questionnaire, these patients will be asked about comprehension of each EmPHasis-10-BR item using the following questions: Was this item understood? What does this item mean? Can this item be explained in different words? Can alternative wording be suggested? A report based on this pilot study will be prepared and reviewed by pulmonary hypertension specialists to identify any additional adjustments that may be required.

After finalization, patients who meet the eligibility criteria will undergo collection of demographic data and information related to health status, comorbidities, symptoms, and complementary test results. Eligible patients will be invited to participate during outpatient clinic visits and will be referred to the Pulmonology Department after completion of the routine appointment to initiate study procedures.

At the first study visit, after signing the written informed consent form, the EmPHasis-10-BR questionnaire will be administered by a trained interviewer. Test-retest reliability will be assessed through an in-person reassessment conducted 7 to 14 days later at the Pulmonology Department. During this second visit, after administration of the EmPHasis-10-BR, participants will also complete the CAMPHOR questionnaire, the SF-36 questionnaire and the Fried Frailty Phenotype criteria.

A third contact will be conducted by telephone 7 to 14 days after Visit 2 to validate the questionnaire for remote administration.

In addition, routine clinical data used for pulmonary hypertension risk stratification will be collected through medical record review, in accordance with standard outpatient care at the Pulmonary Circulation Clinic. These data will include laboratory tests, echocardiography, six-minute walk test, cardiopulmonary exercise testing, cardiac magnetic resonance imaging, and right heart catheterization results.

All collected data will be entered into a Google Sheets® database by a single investigator.

STUDY VARIABLES Independent variables: demographic data; smoking history and smoking index; anthropometric measurements; comorbidities; medications; NYHA functional class; pulmonary hypertension etiology and group; ESC risk stratification; complementary test results.

Dependent variable: EmPHasis-10-BR score.

STATISTICAL ANALYSIS Quantitative variables will be expressed as mean ± standard deviation or median and interquartile range, according to data distribution. The Shapiro-Wilk test will be used to assess normality. Qualitative variables will be expressed as percentages, and 95% confidence intervals will be calculated when appropriate.

Internal consistency of the EmPHasis-10-BR will be evaluated using Cronbach's alpha. Values below 0.7 will be considered indicative of insufficient internal consistency. Item-total correlations are expected to range between 0.2 and 0.8.

Test-retest reliability will be assessed by correlating EmPHasis-10-BR scores obtained at different time points (Visit 1 and Visit 2), assuming no clinical change occurred between administrations. A minimum correlation coefficient of 0.85 will be required to indicate adequate reliability, using Spearman's correlation test. A similar analysis will be performed comparing in-person (Visit 2) and remote (Visit 3) administrations.

Additionally, EmPHasis-10-BR scores will be compared with general and disease-specific quality-of-life questionnaires validated in Portuguese, namely the 36-item Short Form Health Survey (SF-36) and the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR).

All statistical tests will be two-tailed, and p values < 0.05 will be considered statistically significant. Data will be analyzed using the Statistical Package for the Social Sciences (SPSS), version 18.0 for Windows (SPSS Inc., Chicago, IL, USA).

RISKS The potential risks associated with participation in this study include unauthorized disclosure or identification of patient-related data. However, all measures to ensure confidentiality will be adopted. The investigators commit to preserving participants' privacy throughout the study and to using all collected information solely for the execution of this research project.

BENEFITS The expected benefits of this study include the validation of the EmPHasis-10 questionnaire for use in Brazilian Portuguese, aiming to standardize clinical assessment and generate reliable data for longitudinal follow-up. Additionally, the instrument may support non-invasive, low-cost risk stratification and prognostic assessment, with potential applicability across different levels of healthcare.