High levels of healthcare utilization prior to diagnosis in idiopathic pulmonary arterial hypertension support the feasibility of an early diagnosis algorithm: the SPHInX project

Abstract

Idiopathic pulmonary arterial hypertension (iPAH) is a rare progressive, life-shortening disease, usually diagnosed at an advanced stage. We hypothesize that patients with iPAH exhibit patterns of health-seeking behavior before diagnosis that will allow the development of earlier identification tools. The Sheffield Pulmonary Hypertension IndeX (SPHInX) project aims to develop a predictive algorithm based on routinely collected healthcare resource utilization (HCRU) data. This report focuses on the initial feasibility of the project, examining whether Hospital Episode Statistics (HES) data from the National Health Service in England have sufficient richness to support the development of an early diagnosis algorithm. This is a two-stage study. First, hospital interactions during 2009-2014 captured in HES data identified 127,815 adult patients with pulmonary hypertension (PH) ICD-10 codes, containing a probable iPAH cohort with incidence and demographics similar to the reported literature. HCRU was high in the three years before diagnosis. Second, to examine HCRU in patients with a confirmed iPAH diagnosis, we built the SPHInX dataset incorporating all patients investigated for suspected PH in the Sheffield Pulmonary Vascular Disease Unit during 2008-2016 (n = 6674). For the SPHInX dataset, data could be linked to HES in 98.6% of cases and patients with confirmed iPAH had similar levels of pre-diagnosis HCRU. In conclusion, patients with probable iPAH identified using HES and patients with confirmed iPAH have high levels of HCRU for several years before diagnosis. Artificial intelligence models will now be used to develop the SPHInX algorithm to screen for undiagnosed iPAH in the general population.

Screening strategies for pulmonary arterial hypertension

“…Over the last two decades, there have been significant advances in the treatment of PAH and a more than doubling of survival 24 , 25 but the time from symptom onset to diagnosis remains unchanged at around 2 years 26 . A recurring theme of studies is the frequent interaction with healthcare services prior to diagnosis and missed opportunities for earlier diagnosis, particularly in patients with idiopathic PAH (IPAH) 27 . Initial PAH symptoms are non-specific and include breathlessness on exertion, fatigue, chest tightness similar to angina, palpitations, dizziness, and ankle swelling in advanced disease 1 .…”

Editorial: Pulmonary Hypertension: Mechanisms and Management, History and Future

“…Intuitively, earlier diagnosis and institution of treatment may slow vascular remodeling and improve outcomes. However, despite increased awareness and the availability of new therapies the time from symptom onset to diagnosis remains unchanged at 2-3 years, suggesting that new approaches need to be taken to improve disease detection (5). This highlights the importance of identifying easily performed and scalable tests that can be deployed in the investigative pathway.…”

“…Despite advances in treatment and increasing awareness of the disease over the last 2-3 decades, many patients arrive at specialist centers after a significant diagnostic delay (16). While not reviewed in this topic, recent work from our center has sought to improve this issue by highlighting the potential use of artificial intelligence to interrogate routine healthcare data in order to identify patients at high risk of idiopathic PAH at an earlier stage (5). Furthermore, the topic only alludes to the use of novel tools to phenotype these high-risk patients.…”

Expertentreffen COPD: Lunge und Herz – ziemlich oft gemeinsam krank!

“…Ein weiterer Ansatz zur Frühdiagnostik von Patienten mit idiopathischer PAH könnte sich aus der Auswertung von Krankenkassendaten ergeben. Bewegungsmuster in Bezug auf Zahl und Art der Arztkontakte können dazu genutzt werden, um PH-Patienten früher zu identifizieren [113]. In einer Analyse aus England hatten Patienten mit iPAH in den 3 Jahren vor Diagnosestellung im Durchschnitt 23 Krankenhauskontakte (ambulant oder stationär), mit deutlichem Anstieg im letzten Jahr vor der Diagnose.…”