Background and aim Pulmonary Arterial Hypertension (PAH) is a rare progressive disease leading to right heart failure and premature death with a prognosis worse than many cancers. Despite this there is very little published data on the disease trajectory during the terminal phase and how hospital palliative care services are accessed. The aim was to assess the reasons for referral, patient outcomes and challenges encountered in one of the largest Pulmonary Vascular Disease Units (PVDU) in Europe serving a population of in excess of 15 million.

Methodology We performed a retrospective review of consecutive patients referred to a Hospital Palliative Care Team (HPCT) from the Sheffield PVDU between 2011 and 2016. Information was extracted from an electronic patient database (Infoflex) capturing details of referral, diagnosis, assessment and HPCT interventions.

Results 30 patients (16 Female), median age 65.6 (range 18–86) were referred; 73% had ECOG performance status 4. The primary reasons for referral were symptom control (mainly breathlessness 71%) and management of end of life care. Given the quaternary nature of the PAH service, 25/30 patients were from outside our local area. During follow-up, 22/30 patients died. 14 patients died during their hospital admission (mean time to death from referral to HPCT 7 days (range 0–27)). For 8 patients the HPCT facilitated and achieved a preferred place of death (3 to local hospice and 5 to own home). The biggest challenge was coordinating end of life care in timely manner due to the geographic locations of patients.

Conclusion Patients are often referred very late in the terminal phase of their disease process. Whilst the specialist PAH team provide basic symptom control and support, earlier referral and proactive involvement of the HPCT as part of a multidisciplinary team would help address advanced care planning, complex symptom control and end of life care.