Pulmonary Hypertension
Conditions
Keywords
Pulmonary Hypertension, Six-minute walking test, NT-proBNP
Brief summary
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.
Detailed description
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.
Interventions
None listed
Sponsors
Chinese University of Hong Kong
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
19 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Patients age \> 18 with echocardiographic confirmation of increased pulmonary pressure (eg. estimated RVSP \>40mmHg, evidence of RV dilatation etc), and no apparent reversible causes. * Patients with reasonable pre-morbids and is able to attend ambulatory clinic.
Exclusion criteria
* Inability to provide valid consent by the patient or his legal guardian
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| All-cause mortality | 3-monthly | All-cause mortality |
| WHO functional class | 3-monthly | Change in functional class (class I as the mildest to class IV as more severe) as measured by WHO functional class over times |
| EMPASIS-10 questionnaires | 3-monthly | 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 |
| pulmonary hypertension related mortality | 3-monthly | pulmonary hypertension related mortality |
| Borg Rating of perceived exertion (RPE) scale | 3-monthly | 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 |
| The 5-level EQ-5D version (EQ-5D-5L) | 3-monthly | 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 |
| The Modified Medical Research Council (mMRC) Dyspnea scale | 3-monthly | 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 |
| Pulmonary Embolism Quality of Life Questionnaire (PEmb-QoL) | 3-monthly | Change in disease-specific health-related quality of life (QoL) as measured by PEmb-QoL which use to access QoL following PE |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| clinical parameters | 3-monthly | Change in physical parameters |
| physical parameters | 3-monthly | Change in physical parameters |
| laboratory parameters | 3-monthly | Change in laboratory parameters |
| daily activity level | 3-monthly | Change in daily activity level |
| Natriuretic Peptide Tests (NT-proBNP) level | 3-monthly | Change from baseline in log-transformed NT-proBNP level over time |
| Six-minute walking test (6MWT) | 3-monthly | Change in Six-minute walking test (6MWT) |
| Right Ventricular Systolic Pressure (RVSP) as echocardiographic parameters | 3-monthly | Change in RVSP as echocardiographic parameters |
| hemodynamic parameters | 3-monthly | Change in hemodynamic parameters |
| PH specific medications number and dosage | 3-monthly | 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. |
| costs of all services, medications and tests | 3-monthly | 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 |
Countries
Hong Kong
Outcome results
None listed