Exploring the patient perspective in pulmonary hypertension

Abstract

The global impacts of pulmonary hypertension (PH) were formally recognised in 1973 at the 1st World Health Organization meeting dedicated to primary pulmonary hypertension, held in Geneva. Investigations into disease pathogenesis and classification led to the development of numerous therapies over the ensuing decades. While the impacts of the disease have been lessened due to treatments, the symptoms and adverse effects of PH and its therapies on patients' wellbeing and mental health remain significant. As such, there is a critical need to enhance understanding of the challenges patients face on a global scale with respect to care access, multidimensional patient support and advocacy. In addition, thoughtful analysis of the potential benefits and utilisation of mechanisms for the incorporation of patient-reported outcomes into diagnosis and treatment plans is needed. A summary of these areas is included here. We present a report of global surveys of patient and provider experiences and challenges regarding care access and discuss possible solutions. Also addressed is the current state of PH patient associations around the world. Potential ways to enhance patient associations and enable them to provide the utmost support are discussed. A summary of relevant patient-reported outcome measures to assess health-related quality of life in PH is presented, with suggestions regarding incorporation of these tools in patient care and research. Finally, information on how current global threats such as pandemics, climate change and armed conflict may impact PH patients is offered, along with insights as to how they may be mitigated with advanced contingency planning.

FIGURE 1

Distribution of patient associations throughout the world with key organisation attributes noted where known. Countries with a patient association are shaded. Patient associations not part of a larger umbrella organisation by primary membership are listed individually. For a comprehensive international listing of patient associations that is updated over time, refer to: https://phassociation.org/international/. Other organisations and foundations around the world that do not meet the formal criteria of a patient association have also developed research and advocacy efforts to support the pulmonary hypertension (PH) cause. An exhaustive list of all is beyond the scope of this work, but recognition of their presence and impact is imperative. HTAPQ: Hypertension Artérielle Pulmonaire Québec; PHA: Pulmonary Hypertension Association.

FIGURE 2

Patient-reported outcome measures (PROMs): potential value and what they measure. Included is a table summary of commonly used PROMs in routine clinical care and research for pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) [, , –59]. HRQoL: health-related quality of life; PH: pulmonary hypertension; MCID: minimal clinically important difference; CAMPHOR: Cambridge Pulmonary Hypertension Outcome Review; PAH-SYMPACT: Pulmonary Arterial Hypertension – Symptoms and Impact Questionnaire; PAHSIS: Pulmonary Arterial Hypertension Symptom and Interference Scale; EQ-5D: EuroQol 5-dimension; SF-36: 36-Item Short-Form Health Survey questionnaire; 6MWD: 6-min walk distance; FC: functional class; BNP: brain natriuretic peptide, PVR: pulmonary vascular resistance; D_LCO: diffusion capacity of the lung for carbon monoxide. ^#: PAH and CTEPH.

FIGURE 3

Overview of various factors and perspectives affecting and informing the pulmonary hypertension patient's experience of living with the disease. PROMs: patient-reported outcome measures.