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Di Nora et al. Vessel Plus 2022;6:46 https://dx.doi.org/10.20517/2574-1209.2021.126 Page 3 of 7

Table 1. The relevant results in HTx patients affected by AL and ATTR amyloidosis
N of patients AL/ATTR Comments
Grogan et al. [44] 23 AL Five-year survival of 43% vs. 85% for non-amyloid patients
[24]
Rosenbaum et al. 7 ATTR wt One non-amyloid-related death
[45]
Barrett et al. 31 13 AL No differences in survival
18 ATTR
[46]
Mignot et al. 8 AL 75% alive (2.2 years of follow-up)
Kristen et al. [21] 12 NA One- and three-year survival rates similar to non-amyloid transplant patients
[32]
Kristen et al. 48 32 AL Median survival of 61% after 3.5 years
16 ATTR
HTx: Heart transplantation, AL: light-chain.

Heart Transplantation in AL Amyloidosis
The introduction of ASCT after HTx in AL CA patients has changed the scenario of HTx availability in this
subgroup of patients. The first experiences were limited to a few cases. Roiget al. reported sequential
[26]
HTx/ASCT in five patients with AL cardiac amyloidosis: 2/5 died from progressive amyloidosis and 3/5 are
alive, including one who developed progressive plasma cell dyscrasia that was successfully treated with high-
[27]
dose corticosteroids. Lacyet al. from the Mayo Clinic published a series of 11 patients undergoing
sequential HTx/ASCT, reporting that 2/11 patients died from transplant-related toxicity and 3/11 died from
progressive amyloidosis. The largest series of patients transplanted for CA was published in June 2021 from
the United Network for Organ Sharing registry from 1987 to 2018, where the authors reported 313 patients
affected by CA who underwent isolated HTx, with encouraging results .
[28]

Italian data
For the first time in Italy, some data on HTx in CA patients were published, showing that sequential HTx-
ASCT is feasible and assuring a good survival in highly selected AL CA patients, with satisfactory early and
late outcomes . Thus, in this paper, 36 amyloidosis patients are included from 2009 to 2019. Of these, nine
[29]
had cardiac involvement, 7/9 due to AL amyloidosis and 2/9 due to wild-type TTR amyloidosis. All were
survivors during the wait for HTx. ASCT was performed in 6/7 AL CA patients, with a median time of six
months after HTx. Survival was 88% and 66% at one and five years, respectively.

Satisfying results in this peculiar disease could be reached only with specific follow-up programs after HTx-
ASCT to anticipate the potential manifestations of CA over time . Accordingly, some different consensus
[29]
criteria have been defined . Patients who have a total remission of the disease with no evidence of
[30]
monoclonal immunoglobulins in serum or urine and normal serum free light chains reach the best results.
Conversely, a partial response consistent in a reduction of 50% or more in concentrations of aberrant free
light chains is not satisfactory . It is known that renal dysfunction and gastrointestinal amyloid
[31]
[32]
involvement may be associated with a worse prognosis and a higher rate of complications after ASCT . For
these reasons, it is mandatory to deeply evaluate all candidates for any gastrointestinal, hepatic, or renal
involvement, including severe neuropathy or coagulopathy.

Another crucial point in this setting is related to the time of ASCT after HTx. Lacy et al. suggested to
[27]
pursue ASCT in a time near to six months after HTx: sooner it may be difficult if the patient continues to
require intensive immunosuppression, and later it may be too late for the risk of amyloidosis progression in
other organs. This time-control strategy between HTx and ASCT could be another point of improvement in
the survival of amyloidotic patients after HTx, in contrast to the previous analysis .
[33]