Priorities for pulmonary hypertension research: A James Lind Alliance priority setting partnership.

Pulmonary hypertension (PH) is a rare condition associated with significant morbidity and mortality. The priorities for future research in PH according to patients, caregivers, and clinicians have not been established. We performed a James Lind Alliance priority setting partnership in Canada. An initial survey of 249 respondents (76% patients and/or caregivers, 12.5% clinicians) generated 1588 questions, which were combined into 187 summary questions. An evidence check identified 352 systematic reviews and guidelines which were mapped to the summary questions, which determined that 157 summary questions were unanswered by existing research. An interim prioritisation survey (240 respondents, 66% patients and/or caregivers, 18% clinicians) asked respondents to choose which of the 157 summary questions were among the 30-40 most important. In a final workshop patients, caregivers, and clinicians discussed and ranked the top 25 questions from the interim survey to identify the Top 10 PH research priorities. These results will inform researchers and funding bodies about patient, caregiver, and clinician priorities for future research on PH.

Does community size or commute time affect severity of illness at diagnosis or quality of care in a centralized care model of pulmonary hypertension?

BACKGROUND: Centralized care models are often used for rare diseases like pulmonary hypertension (PH). It is unknown how living in a rural or remote area influences outcomes. METHODS: We identified all patients from our PH database who carried a diagnosis of WHO Group 1 or WHO Group 4 PH. Using Canadian postal code data, patients were classified as living in a rural area; or a small, medium or large community size. The commute time from patient residence to our clinic was determined using mapping software. We compared baseline catheterization data according to community size and commute time. At follow up, we evaluated the association between community size and commute time with prognostic parameters of functional class, walk distance and echocardiography. RESULTS: Of the 342 patients identified, 72(21%) patients lived in rural areas, while 26(8%), 49(14%) and 195(57%) resided in small, medium and large population centres, respectively. The commute time was <1 h for 160(47%), 1-3 h for 62(18%), and >3 h for 120(35%). There was no association seen for any catheterization parameter by either community size or commute time. At last follow up, there was no association between any prognostic parameter and community size or commute time. CONCLUSIONS: We found no association between community size or commute time with severity of illness at diagnosis, or markers of prognosis at follow up. This suggests that patients who reside in rural or remote environments are not experiencing deficiencies in care compared to urban patients.