Pulmonary hypertension (PH) is a rare, complex, and progressive disease which defines a group of clinical conditions with abnormal elevation in the pulmonary circulation pressure. There are five types of PH depending on the underlying disease-causing PH. In this study we will focus on Pulmonary Arterial Hypertension (PAH) Group 1 and pulmonary hypertension associated with lung disease (PH-LD) (Group 3).
The number of people suffering PH and especially PH-LD in the UK is unclear. Data from economically developed countries suggest a PAH incidence and prevalence of ~6 and 48 to 55 cases/million adults, respectively. The prevalence of PH-LD in Europe remains largely unknown. Most studies report the prevalence of PH associated with specific LDs and not for the whole Group 3 and are restricted to smaller data sources.
PH is a debilitating disease that impacts all aspects of a patient's daily life, from physical activity, general health, and vitality to emotional and social functioning, and increased risk of death. Although therapies exist to treat patients with PAH, currently there are no approved therapies to treat PH-LD in Europe.
This study aims at increasing our overall understanding of the PAH and PH-LD disease by estimating the epidemiology of the disease and characterizing the burden, treatment patterns and real-world outcomes of patients living with PAH and PH-LD.
Pulmonary hypertension (PH) is a rare, complex, and progressive disease which defines a group of clinical conditions with abnormal elevation in the pulmonary circulation pressure. PH may involve multiple clinical conditions and can complicate most of the cardiovascular and respiratory diseases. PH can be classified into five different clinical groups according to the underlying pathophysiology and clinical presentation. In this study we are targeting patients with pulmonary arterial hypertension (PAH) (Group 1), pulmonary hypertension associated with lung disease and/or hypoxia (PH-LD) (Group 3) and subset of Group 3, pulmonary hypertension associated with interstitial lung disease (PH-ILD). The objective of this study is to characterize the epidemiology, the burden of disease, describe treatment patterns and the real-world outcomes of patients with PH-LD and PH-ILD. Additionally, we will also characterize the treatment patterns of patients with PAH. Data from 1 January 2014 to 31 December 2021 will be obtained from the CPRD linked to the National Health Service (NHS) Digital Hospital Episode Statistics database. These data sources provide patient care activity from a network of GP practices across the UK and patient events occurring in NHS England hospitals. An algorithm to identify adult patients with PAH, PH-LD and PH-ILD using electronic medical record databases has been developed based on previous studies and validated with a scientific committee composed by a group of PH experts. The PAH algorithm will integrate PH diagnosis codes, PAH-associated procedures and PAH-therapies, while the Group 3 PH identification algorithm will incorporate pulmonary hypertension (PH) and lung diseases (LD) diagnosis codes. The analyses will be descriptive. Treatment patterns will include prescribed drugs and procedures. Real world outcomes will include inpatient admissions, outpatient visits and Accident and Emergency (A&E) attendances. Associated costs, will be calculated using national tariffs inflation-adjusted to 2021.
Age; Sex; Patient baseline characteristics; Incidence; Prevalence; Pulmonary Hypertension diagnosis; Lung-Disease diagnosis; Treatment patterns; Disease burden; Economic burden; Comorbidities; Mortality; Reason for Hospitalization; Hospitalisation rate; Length of stay; Healthcare resource utilisation; Health care costs
Aurélie CHEKROUN MARTINOT - Chief Investigator - Alira Health SAS (France)
Catia Proenca - Corresponding Applicant - Alira Health SAS (France)
GABRIELA BACCHINI JEANNERET - Collaborator - Ferrer Internacional, S.A.
Maria Pini - Collaborator - Alira Health SAS (France)
Nicoleta Petrica - Collaborator - Alira Health SAS (France)
Stephen John Wort - Collaborator - Royal Brompton Hospital
HES Accident and Emergency;HES Admitted Patient Care;HES Outpatient;ONS Death Registration Data