Kiely, D.G. orcid.org/0000-0003-0184-6502, Lawrie, A. orcid.org/0000-0003-4192-9505 and Humbert, M. (2019) Screening strategies for pulmonary arterial hypertension. European Heart Journal Supplements, 21 (Supplement_K). K9-K20. ISSN 1520-765X
Abstract
Pulmonary arterial hypertension (PAH) is rare and, if untreated, has a median survival of 2–3 years. Pulmonary arterial hypertension may be idiopathic (IPAH) but is frequently associated with other conditions. Despite increased awareness, therapeutic advances, and improved outcomes, the time from symptom onset to diagnosis remains unchanged. The commonest symptoms of PAH (breathlessness and fatigue) are non-specific and clinical signs are usually subtle, frequently preventing early diagnosis where therapies may be more effective. The failure to improve the time to diagnosis largely reflects an inability to identify patients at increased risk of PAH using current approaches. To date, strategies to improve the time to diagnosis have focused on screening patients with a high prevalence [systemic sclerosis (10%), patients with portal hypertension assessed for liver transplantation (2–6%), carriers of mutations of the gene encoding bone morphogenetic protein receptor type II, and first-degree relatives of patients with heritable PAH]. In systemic sclerosis, screening algorithms have demonstrated that patients can be identified earlier, however, current approaches are resource intensive. Until, recently, it has not been considered possible to screen populations for rare conditions such as IPAH (prevalence 5–15/million/year). However, there is interest in the use of artificial intelligence approaches in medicine and the application of diagnostic algorithms to large healthcare data sets, to identify patients at risk of rare conditions. In this article, we review current approaches and challenges in screening for PAH and explore novel population-based approaches to improve detection.
Metadata
Item Type: | Article |
---|---|
Authors/Creators: |
|
Copyright, Publisher and Additional Information: | © The Author(s) 2019. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. |
Keywords: | Pulmonary arterial hypertension; Screening; Diagnosis |
Dates: |
|
Institution: | The University of Sheffield |
Academic Units: | The University of Sheffield > Sheffield Teaching Hospitals |
Funding Information: | Funder Grant number British Heart Foundation BHF-FS/18/52/33808 |
Depositing User: | Symplectic Sheffield |
Date Deposited: | 29 Jan 2020 09:35 |
Last Modified: | 29 Jan 2020 09:35 |
Status: | Published |
Publisher: | Oxford University Press (OUP) |
Refereed: | Yes |
Identification Number: | 10.1093/eurheartj/suz204 |
Related URLs: | |
Open Archives Initiative ID (OAI ID): | oai:eprints.whiterose.ac.uk:155073 |