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

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 Overview

• Status: Enrolling by invitation
• Conditions: Pulmonary Hypertension

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.

• Study Type: Observational
• Enrollment (Estimated): 100

Contacts and Locations

Study Locations

• Hong Kong
  • Hong Kong, Hong Kong
    • Prince of Wales Hospital, Hong Kong, Hong Kong

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Adult; Older Adult
• Accepts Healthy Volunteers: No

• Sampling Method: Probability Sample

Study Population

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.

Description

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

Study Plan

How is the study designed?

Number of groups / cohorts

1

Cohorts and Interventions

Group / Cohort
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

What is the study measuring?

Primary Outcome Measures

Outcome Measure	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	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

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
physical parameters	Change in physical parameters	3-monthly
daily activity level	Change in daily activity level	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

Collaborators and Investigators

• Sponsor: Chinese University of Hong Kong

Study record dates

Study Major Dates

• Study Start (Actual): November 5, 2024
• Primary Completion (Estimated): November 5, 2029
• Study Completion (Estimated): February 5, 2030

Study Registration Dates

• First Submitted: November 20, 2024
• First Submitted That Met QC Criteria: November 21, 2024
• First Posted (Estimated): November 25, 2024

Study Record Updates

• Last Update Posted (Estimated): November 25, 2024
• Last Update Submitted That Met QC Criteria: November 21, 2024
• Last Verified: November 1, 2024

More Information

Terms related to this study

• Keywords: Pulmonary Hypertension; NT-proBNP; Six-minute walking test
• Additional Relevant MeSH Terms: Vascular Diseases; Cardiovascular Diseases; Respiratory Tract Diseases; Lung Diseases; Hypertension; Hypertension, Pulmonary
• Other Study ID Numbers: 2024.290

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No