The UK National Cohort Study of Idiopathic and Heritable Pulmonary Arterial Hypertension ten years on: an Overview and its Future.

Pulmonary Hypertension (PH) affects up to 1% of people globally. Increased understanding of pathophysiology over the last 3 decades has improved treatment options and outcomes for a subset of patients diagnosed with WHO groups 1 and 4 PH. However, advances within other PH groups have been limited. The BHF National Cohort Study has significantly impacted PH care and research. However, its scope has been limited to idiopathic and heritable Pulmonary Arterial Hypertension (PAH). Here we present an overview of the study and our ongoing plans to evolve the cohort to be an international community resource with a broader and more inclusive approach to PH. The Cohort Study, currently, includes 957 PAH patients, 161 relatives, and 139 healthy controls from 9 centres. Longitudinally repeated serum and urine samples have been stored to allow for deep phenotyping of the cohort. A wide range of clinical data for both patients and relatives is available. Additionally, there are multiple nested studies. The study design is described in Figure 1A. Since its inception, the Cohort Study has made key discoveries, some of which are described in figure 1C. As of 2024, the Cohort Study has been amended to include all patients with PH. The structure of the data being collected is additionally changing, as described in figure 1B. Finally, we aim to increase open access to the PH community in 2025. <fig><object-id>erj;64/suppl_68/PA2496/F1</object-id><object-id>F1</object-id><object-id>F1</object-id><graphic></graphic></fig>