Pulmonary Hypertension (PH) is a multifaceted disease and is associated with significant morbidity and mortality if untreated1. Diagnosing PH can be challenging and often delayed2. Multiple international registries included REVEAL3 and COMPERA4 have all demonstrated that diagnostic delay and suboptimal treatment prescription led poor 1 year-survival in patient with PH. In recent years, significant progress has been made in diagnosing, risk stratification, and treatment for patients with PH1. A dedicated PH service has been established in ambulatory clinic to streamline the management of these complex patients. This registry therefore aims to investigate the progression disease and its impact on the quality of life (QoL) of the patients who are managed at the dedicated PH clinic.
Pulmonary Hypertension (PH) is a multifaceted disease and is associated with significant morbidity and mortality if untreated1. Diagnosing PH can be challenging and often delayed2. Multiple international registries included REVEAL3 and COMPERA4 have all demonstrated that diagnostic delay and suboptimal treatment prescription led poor 1 year-survival in patient with PH. In recent years, significant progress has been made in diagnosing, risk stratification, and treatment for patients with PH1. A dedicated PH service has been established in ambulatory clinic to streamline the management of these complex patients. This registry therefore aims to investigate the progression disease and its impact on the quality of life (QoL) of the patients who are managed at the dedicated PH clinic.
Study Type
OBSERVATIONAL
Enrollment
100
Prince of Wales Hospital, Hong Kong, Hong Kong
Hong Kong, Hong Kong
All-cause mortality
All-cause mortality
Time frame: 3-monthly
WHO functional class
Change in functional class (class I as the mildest to class IV as more severe) as measured by WHO functional class over times
Time frame: 3-monthly
EMPASIS-10 questionnaires
Change in disease-specific health-related quality of life (QoL) as measured by EMPASIS-10 questionnaires which is an independent prognostic marker in patients with (I/D/H)PAH or CTD-PAH
Time frame: 3-monthly
pulmonary hypertension related mortality
pulmonary hypertension related mortality
Time frame: 3-monthly
Borg Rating of perceived exertion (RPE) scale
Change in disease-specific health-related quality of life (QoL) as measured by Borg RPE scale which is an outcome measure scale used to gauge one's exercise intensity without the need to rely on physiological parameters
Time frame: 3-monthly
The 5-level EQ-5D version (EQ-5D-5L)
Change in disease-specific health-related quality of life (QoL) as measured by EQ-5D-5L which is for assessing patient health states for clinical and economic appraisal
Time frame: 3-monthly
The Modified Medical Research Council (mMRC) Dyspnea scale
Change in disease-specific health-related quality of life (QoL) as measured by mMRC which use to access stratifies severity of dyspnea in respiratory diseases, particularly COPD
Time frame: 3-monthly
Pulmonary Embolism Quality of Life Questionnaire (PEmb-QoL)
Change in disease-specific health-related quality of life (QoL) as measured by PEmb-QoL which use to access QoL following PE
Time frame: 3-monthly
physical parameters
Change in physical parameters
Time frame: 3-monthly
daily activity level
Change in daily activity level
Time frame: 3-monthly
Natriuretic Peptide Tests (NT-proBNP) level
Change from baseline in log-transformed NT-proBNP level over time
Time frame: 3-monthly
Six-minute walking test (6MWT)
Change in Six-minute walking test (6MWT)
Time frame: 3-monthly
Right Ventricular Systolic Pressure (RVSP) as echocardiographic parameters
Change in RVSP as echocardiographic parameters
Time frame: 3-monthly
hemodynamic parameters
Change in hemodynamic parameters
Time frame: 3-monthly
PH specific medications number and dosage
Change of PH specific medications number and dosage over time, including PDE5 inhibitor, Guanyl Cyclase stimulator, Endothelin Receptor antagonist, prostacyclin receptor agonist, and inhaled prostacyclin.
Time frame: 3-monthly
costs of all services, medications and tests
The costs of all services, medications and tests as estimated with reference to the Hong Kong Government Gazette 2020 and Hong Kong Hospital Authority Drug Formulary 2020
Time frame: 3-monthly
clinical parameters
Change in physical parameters
Time frame: 3-monthly
This platform is for informational purposes only and does not constitute medical advice. Always consult a qualified healthcare professional.
laboratory parameters
Change in laboratory parameters
Time frame: 3-monthly