Case Presentation: WF, an 88-year-old man with advanced, wild-type transthyretin-associated (ATTR) cardiac amyloidosis (on tafamidis) complicated by distal conduction disease and heart failure with preserved ejection fraction, presented with syncope and was admitted to the cardiac critical care unit for symptomatic, bradycardic atrial fibrillation. A continuous dopamine infusion was started with resolution of symptoms and improvement in heart rate (from 30-40bps to 40-60bps). However, attempts to wean from dopamine with midodrine and methylphenidate were unsuccessful, resulting in hypotension and pre-syncope.Palliative care was consulted for support of goals of care. WF expressed a clear preference to prioritize his independence and declined life-prolonging therapies that could adversely affect his quality of life, including resuscitation/intubation. In the absence of facilities or home services able to provide continuous infusions of palliative dopamine, the option of pacemaker placement was revisited. While WF had previously declined pacemaker placement for the purpose of life prolongation, he expressed a willingness to undergo the procedure if it could allow him to return home with the possibility of fewer hospitalizations, despite uncertainty regarding its potential impact on his functional status and quality of life.On hospital day six, WF experienced worsening delirium and he no longer demonstrated capacity to consent for pacemaker placement. His niece/health care proxy arrived from out of town to formulate a new plan of care based on knowledge of his values, which he had previously communicated to the primary and palliative interdisciplinary teams. Based on his preferences for independence and comfort over prolongation of life, a shared decision was made to transfer WF to the inpatient palliative care unit. WF was eventually weaned from dopamine, but did not return to his baseline mental status, and was later discharged to an outpatient hospice facility in alignment with his expressed goals.

Discussion: Despite novel gene-silencing therapies and improved diagnostic techniques, advanced cardiac amyloidosis is associated with poor prognosis and quality of life, including symptoms of syncope and sudden death.(1,2) Compared to immunoglobulin light chain (AL) amyloidosis, ATTR cardiac amyloidosis has been observed to have a more slowly progressive disease course and more favorable survival, with newly proposed staging systems.(3-5) However, there is limited research characterizing the quality of life associated with different stages and treatments of this disease. While some patients may benefit from improved survival with implantable cardioverter-defibrillator (ICD) devices, pacemakers/ICDs have not been shown to improve quality of life in this setting.(6,7)This case demonstrates the importance of a proactive approach to advance care planning for patients with ATTR cardiac amyloidosis. This requires interdisciplinary teamwork and should address a wide range of clinical scenarios, including the role of pacemaker/ICD placement, the palliative use of inotropes (which may impact care settings),(8) and the possibility of progressive functional loss with frequent hospitalizations.

Conclusions: In the absence of evidence-based approaches to advance care planning for patients with advanced ATTR cardiac amyloidosis, palliative care consultation can help to support the development of goal-concordant care plans despite clinical uncertainty.