Insights from the Pulmonary Hypertension Global Patient Survey: challenges in pulmonary hypertension care abound but so do opportunities

Extract

Pulmonary hypertension (PH) exerts a significant burden on patients and healthcare systems globally, affecting an estimated 1% of the world's population and leading to significant morbidity, mortality and cost [1–3]. Over the past several decades, the PH research community has made significant strides in uncovering the molecular mechanisms of PH, in PH drug development and in refining PH phenotyping, among other substantial advancements [4–6]. Yet, outcomes for patients living with PH remain unacceptably poor and significant gaps in PH care delivery persist. Understanding the global state of PH care from the patient perspective is fundamental to improve PH care quality and outcomes by highlighting areas of strength that can be further capitalised and revealing vulnerable areas that need to be bolstered worldwide. While patient views on PH care have been gathered from individual countries and regions, to date, a large-scale global collection of patient perspectives has not been achieved.

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Insights from the Pulmonary Hypertension Global Patient Survey https://bit.ly/4m5MOaS

Pulmonary hypertension (PH) exerts a significant burden on patients and healthcare systems globally, affecting an estimated 1% of the world's population and leading to significant morbidity, mortality and cost [1–3]. Over the past several decades, the PH research community has made significant strides in uncovering the molecular mechanisms of PH, in PH drug development and in refining PH phenotyping, among other substantial advancements [4–6]. Yet, outcomes for patients living with PH remain unacceptably poor and significant gaps in PH care delivery persist. Understanding the global state of PH care from the patient perspective is fundamental to improve PH care quality and outcomes by highlighting areas of strength that can be further capitalised and revealing vulnerable areas that need to be bolstered worldwide. While patient views on PH care have been gathered from individual countries and regions, to date, a large-scale global collection of patient perspectives has not been achieved.

The PH Global Patient Survey (GPS), designed and disseminated by the Pulmonary Vascular Research Institute (PVRI)'s Patient Engagement and Empowerment Group, begins the important work of characterising the global landscape of PH care from the patient viewpoint. Investigators of the PH GPS met regularly with key stakeholders including patients and patient advocates to co-design a patient-centred survey based on stakeholder priorities. They then partnered with nearly 50 PH patient organisations to distribute the survey to patients with all PH groups across 90 countries and 24 languages, garnering responses from >3500 patients or their proxies. In this issue of ERJ Open Research, Newman et al. [7] present the first findings from the PH GPS and report on a range of themes, including access to specialty care, patient self-monitoring, telemedicine, research participation and use of patient-reported outcome measures (PROMs).

Perhaps unsurprising, though nevertheless discouraging, patients noted a number of significant barriers to receiving PH care, including 1) geographic barriers (half of participants travelled more than an hour by car to reach a PH centre with the majority noting difficulty attending in-person appointments), 2) financial barriers (over a third of participants had no or only partial coverage of their PH-related healthcare costs), and 3) limited access to rehabilitation and disability-related services. Furthermore, despite the vast majority of participants being under the care of a specialist PH centre, two-thirds of eligible participants with group 1 PH were not offered genetic testing, 40% of patients with group 4 PH did not receive mechanical intervention and nearly three-quarters of all patients had never participated in a research trial. PROMs were also underutilised to guide clinical management, with only 20% of participants having ever completed a PROM and only 7% of these perceiving that the PROM had influenced their care. This is despite the known significant impact PH has on patients’ health-related quality of life reported here and in other work, and despite prior work showing that PROMs may add prognostic value and allow for more patient-centred clinical assessments [8–11]. Finally, the PH GPS identified significant global disparities in PH care, with fewer patients from the Global South participating in research trials, being offered genetic testing, being on parenteral therapy or having financial coverage of their healthcare costs (figure 1).

FIGURE 1.

Maps demonstrating global disparities in a) research participation, b) access to genetic testing, c) access to parenteral therapy and d) coverage for pulmonary hypertension (PH) healthcare costs per data from the PH Global Patient Survey. Countries shaded in white did not have any survey participants. a) Percentage of survey participants who had taken part in a research trial, by geographic region. b) Percentage of survey participants with group 1 PH who had undergone genetic testing, by geographic region. c) Percentage of survey participants with group 1 PH who were on parenteral therapy, by geographic region. Fewer than five participants from Africa or Oceania contributed data on parenteral therapy, so the exact percentage could not be calculated for these regions. d) Percentage of survey participants with full coverage of PH healthcare costs, by geographic region. Fewer than five participants from Africa contributed data on full healthcare coverage, so the exact percentage could not be calculated for this regions. Map created with ArcGIS.

Despite the plethora of challenges identified by the PH GPS, this work also highlights clear opportunities to improve the quality and access of PH care globally and to mitigate geographic disparities. For example, while participants noted that most of their PH care is conducted in person, >80% of participants reported being open to receiving virtual care. Improving the uptake of virtual visits would help reach rural patients and those with transportation challenges, and could be an important component of PH care in areas with limited access to PH specialist centres [12, 13]. Furthermore, half of participants are already self-monitoring their health status, often through digital means, though are not always reporting this data to their PH teams. This data could complement traditionally collected clinical parameters to improve the quality and safety of PH care if better utilised [14, 15]. Finally, while few participants have participated in research trials, the overwhelming majority would participate if given the opportunity. As the pace of drug discovery in PH accelerates, tapping into this group of willing research participants will be vital to conduct clinical trials across a range of settings and in more diverse patient populations [16].

There are important limitations to the PH GPS to note. More than two-thirds of participants were from Europe and North America, with limited representation of patients from Africa, Asia and Oceania. Furthermore, as the survey was administered solely online and disseminated primarily through patient organisations, patients without reliable computer or internet access and those not currently engaged in patient organisations are likely underrepresented in the sample. Indeed, most participants had group 1 PH, with underrepresentation of patients with the more prevalent groups 2 and 3 PH. Additionally, the finding that >80% of patients were under the care of a PH specialist centre is in contrast with real-world studies in the USA showing this rate to be much lower [13, 17]. It is therefore likely that the barriers to PH care identified in this study are even greater for those in the Global South, those in other rural areas and those not currently receiving care from a PH centre or engaged with a patient organisation.

Importantly, the PVRI intends to continue this vital work with future iterations of the PH GPS, including deliberate targeting of patients underrepresented in this study (i.e. patients with PH Groups 2, 3 and 5, and those from the Global South). As was clear from this study, cross-border partnerships and collaboration will be key to identify these harder-to-reach populations. Partnerships with established global PH registries, such as PVRI's GoDeep Registry, may facilitate future iterations of the PH GPS to reach underrepresented populations [18].

The investigators and contributors to the PH GPS should be commended for initiating the immense yet critical effort of gathering global patient perspectives on PH care. The findings of this work will no doubt spark new ideas and partnerships in clinical care, research and implementation to improve PH care and outcomes for patients worldwide.