Roles of specific drug therapies for severe pulmonary arterial hypertension in palliative care: a case report.

Pulmonary arterial hypertension (PAH) is an uncommon but potentially life-threatening illness. The elevated pulmonary arterial blood pressure will lead to right heart failure and even cardiopulmonary collapse if it is severe. Apart from general supportive measures, pulmonary hypertension-specific therapies (PHST) are available to slow down disease progression, but they are not curative. According to previous studies, many patients with severe PAH often had significant physical symptoms and psychological distress for both themselves and their families at the end of life. Moreover, they would have repeated admissions due to physical deterioration and decline in functioning associated with worsening in cardiac function. Some of these patients might suffer from unnecessary aggressive interventions and resuscitation because of lack of advance care planning discussion. As such, Palliative Care (PC) would be an important element as part of the overall management. Hereby, we reported a patient with severe PAH and schizoaffective disorder achieved good outcome from the use of combination PHST and PC input. Her physical and psychosocial needs could be addressed well by PC team. This case report showed the collaborative work between PC and parent teams. While parent team was able to manage the medical conditions related to PAH, however the physical and psychosocial needs should be addressed by PC team. More importantly, the PC team has essential roles of providing advance care planning discussions with the patient and help to reduce unnecessary interventions. Furthermore, PC would coordinate the community service to support her while staying at home. Roles of PHST will also be discussed in this case. Clinicians must take into account possible hemodynamic effects of any palliative intervention for PAH.