United network for organ sharing outcomes after heart transplantation for al compared to ATTR cardiac amyloidosis

Clin Transplant. 2020 Oct;34(10):e14028. doi: 10.1111/ctr.14028. Epub 2020 Jul 24.

Abstract

Light-chain (AL) cardiac amyloidosis (CA) has a worse prognosis than transthyretin (ATTR) CA. In this single-center study, we compared post-heart transplant (OHT, orthotopic heart transplantation) survival for AL and ATTR amyloidosis, hypothesizing that these differences would persist post-OHT. Thirty-nine patients with CA (AL, n = 18; ATTR, n = 21) and 1023 non-amyloidosis subjects undergoing OHT were included. Cox proportional hazards modeling was used to evaluate the impact of amyloid subtype and era (early era: from 2001 to 2007; late era: from 2008 to 2018) on survival post-OHT. Survival for non-amyloid patients was greater than ATTR (P = .034) and AL (P < .001) patients in the early era. One, 3-, and 5-year survival rates were higher for ATTR patients than AL patients in the early era (100% vs 75%, 67% vs 50%, and 67% vs 33%, respectively, for ATTR and AL patients). Survival in the non-amyloid cohort was 87% at 1 year, 81% at 3 years, and 76% at 5 years post-OHT. In the late era, AL and ATTR patients had unadjusted 1-year, 3-year, and 5-year survival rates of 100%, which was comparable to non-amyloid patients (90% vs 84% vs 81%). Overall, these findings demonstrate that in the current era, differences in post-OHT survival for AL compared to ATTR are diminishing; OHT outcomes for selected patients with CA do not differ from non-amyloidosis patients.

Keywords: amyloid; cardiomyopathy; heart transplant; light-chain; transthyretin.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Amyloid Neuropathies, Familial* / surgery
  • Amyloidosis*
  • Cardiomyopathies* / etiology
  • Heart Transplantation*
  • Humans
  • Prealbumin
  • Prognosis
  • Survival Rate

Substances

  • Prealbumin