Outcomes in Patients With Cardiac Amyloidosis and Implantable Cardioverter-defibrillator

Eun-Jeong Kim; Benjamin B. Holmes; Shi Huang; Ricardo Lugo; Asad Al Aboud; Stacey Goodman; Rebecca R. Hung; David Slosky; William G. Stevenson; Gregory F. Michaud; Roy M. John


Europace. 2020;22(8):1216-1223. 

In This Article

Abstract and Introduction


Aims: Cardiac amyloidosis (CA) is associated with increased mortality due to arrhythmias, heart failure, and electromechanical dissociation. However, the role of an implantable cardioverter-defibrillator (ICD) remains unclear. We conducted case-control study to assess survival in CA patients with and without a primary prevention ICD and compared outcomes to an age, sex, and device implant year-matched non-CA group with primary prevention ICD.

Methods and Results: There were 91 subjects with CA [mean age= 71.2 ± 10.2, female 22.0%, 49 AL with Mayo Stage 2.9 ± 1.0, 41 transthyretin amyloidosis (ATTR), 1 other] followed by Vanderbilt Amyloidosis centre. Patients with ICD (n = 23) were compared with those without (n = 68) and a non-amyloid group with ICD (n = 46). All subjects with ICD had implantation for primary prevention. Mean left ventricular ejection fraction was 36.2% ± 14.4% in CA with ICD, 41.0% ± 10.6% in CA without ICD, and 33.5% ± 14.4% in non-CA patients. Over 3.5 ± 3.1 years, 6 (26.1%) CA, and 12 (26.1%) non-CA subjects received ICD therapies (P = 0.71). Patients with CA had a significantly higher mortality (43.9% vs. 17.4%, P = 0.002) compared with the non-CA group. Mean time from device implantation to death was 21.8 months in AL and 22.8 months in ATTR patients. There was no significant difference in mortality between CA patients who did and did not receive an ICD (39.0% vs. 46.0%, P = 0.59).

Conclusions: Despite comparable event rates patients with CA had a significantly higher mortality and ICDs were not associated with longer survival. With the emergence of effective therapy for AL amyloidosis, further study of ICD is needed in this group.


In systemic amyloidosis, cardiac involvement is associated with a poor prognosis. Patients with light chain (AL) amyloidosis have a median survival of 13 months without treatment and 6 months from the onset of heart failure symptoms.[1] Unlike the AL-type, transthyretin amyloidosis (ATTR) tends to have more protracted course with median survival of 60 months following heart failure symptoms if untreated.[2,3] Despite a significant impact on mortality following the onset of cardiac symptoms, little is known about the utility of implantable cardioverter-defibrillators (ICDs) in this population. Case reports suggest that sudden death is the most common mode of death in patients with cardiac amyloidosis (CA).[4] However, recently Lin et al.[5] reported that there was no survival benefit in those with an ICD despite a high rate of appropriate ICD therapies, especially in patients with AL-type amyloidosis. There is considerable debate regarding the role of ICD as a primary prevention strategy because of the limited survival of these patients after a diagnosis of heart failure. In addition, patients may not display systolic dysfunction until later stages of the disease when their prognosis is markedly limited. In this study, we sought to investigate the role of ICD for primary prevention of sudden death in patients with CA and compared them to an age matched non-amyloid population with an ICDs.