Evaluation of the Progression of Disease and Health-related Quality of Life in Patients With Pulmonary Hypertension: A Local Clinic-based Registry

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.

Recruitment & Eligibility

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

Primary Outcome Measures

Name	Time	Method
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 Outcome Measures

Name	Time	Method
physical parameters	3-monthly	Change in physical 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
clinical parameters	3-monthly	Change in physical parameters
laboratory parameters	3-monthly	Change in laboratory parameters

Locations (1): Prince of Wales Hospital, Hong Kong, Hong Kong
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Hong Kong, Hong Kong