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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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Patients at the Prince of Wales Hospital ambulatory cardiac clinic | Patients age > 18 with echocardiographic confirmation of increased pulmonary pressure (eg. estimated RVSP >40mmHg, evidence of RV dilatation etc) and able to attend at the Prince of Wales Hospital ambulatory cardiac clinic |
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| Measure | Description | Time Frame |
|---|---|---|
| All-cause mortality | All-cause mortality | 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 | 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 | 3-monthly |
| pulmonary hypertension related mortality | pulmonary hypertension related mortality | 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 | 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 | 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 |
| Measure | Description | Time Frame |
|---|---|---|
| physical parameters | Change in physical parameters | 3-monthly |
| daily activity level | Change in daily activity level | 3-monthly |
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Inclusion Criteria:
Exclusion Criteria:
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Patients with echocardiographic confirmation of increased pulmonary pressure referred for evaluation and management of PH at the Prince of Wales Hospital ambulatory cardiac clinic will be recruited into this registry. Patients' consent will be sought from either the patients or their legal guardian should the patients deemed incapable to consent.
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Prince of Wales Hospital, Hong Kong, Hong Kong | Hong Kong | Hong Kong |
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| 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 | 3-monthly |
| Natriuretic Peptide Tests (NT-proBNP) level | Change from baseline in log-transformed NT-proBNP level over time | 3-monthly |
| Six-minute walking test (6MWT) | Change in Six-minute walking test (6MWT) | 3-monthly |
| Right Ventricular Systolic Pressure (RVSP) as echocardiographic parameters | Change in RVSP as echocardiographic parameters | 3-monthly |
| hemodynamic parameters | Change in hemodynamic parameters | 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. | 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 | 3-monthly |
| clinical parameters | Change in physical parameters | 3-monthly |
| laboratory parameters | Change in laboratory parameters | 3-monthly |
| ID | Term |
|---|---|
| D006976 | Hypertension, Pulmonary |
| ID | Term |
|---|---|
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
| D006973 | Hypertension |
| D014652 | Vascular Diseases |
| D002318 | Cardiovascular Diseases |
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