Evaluating Outcomes of ACM Patients Implanted with Durable MCSDs

By Justin Grodin, M.D., M.P.H., Assistant Professor of Internal Medicine, and Katherine Michelis, M.D., Assistant Professor of Internal Medicine

Amyloid cardiomyopathy (ACM) is an infiltrative disease, with the majority of cases attributable to aggregation of immunoglobulin light chains (AL) or misfolded transthyretin (ATTR). Due to enhanced, noninvasive diagnostic approaches and recently available therapeutic agents, clinicians are increasingly seeking, and making, the diagnosis of ACM.

Despite these advancements, however, some patients with ACM will progress to advanced heart failure or be diagnosed late in their disease course. Heart transplantation is an available option for highly selected patients with advanced ACM, but some patients may be too ill to wait for a donor; others may simply not be good candidates for heart transplantation. For such patients, mechanical circulatory support devices (MCSDs) are a consideration, but prior studies of their use in these patients have been limited to smaller populations with limited generalizability.

At the 2020 AHA Scientific Sessions, we presented a study, simultaneously published in Circulation Heart Failure, in which we evaluated outcomes of ACM patients implanted with durable MCSDs who were enrolled in the INTERMACS (Interagency Registry for Mechanically Assisted Circulatory Support) database – a large multicenter North American registry. Compared with patients who have dilated cardiomyopathy or other types of restrictive cardiomyopathy, those with ACM received more biventricular support and had a higher incidence of death, even when considering the competing potential for heart transplantation. ACM patients also suffered from frequent adverse events early after implantation. Specifically, patients with ACM experienced higher frequencies of major bleeding, neurologic events, and renal dysfunction when compared to patients with other types of cardiomyopathy.

Overall, these data – from the largest, multicenter cohort of ACM patients implanted with MCSDs to date – highlight concerns with the use of durable MCSDs for patients with advanced ACM.