Emerging therapy in light-chain and acquired transthyretin-related amyloidosis: an Italian single-centre experience in heart transplantation.
Amyloid Neuropathies, Familial
/ complications
Cardiomyopathies
/ diagnosis
Female
Graft Rejection
/ diagnosis
Heart Transplantation
/ adverse effects
Humans
Immunoglobulin Light-chain Amyloidosis
/ complications
Italy
/ epidemiology
Male
Middle Aged
Outcome and Process Assessment, Health Care
Patient Selection
Postoperative Complications
/ diagnosis
Prognosis
Renal Insufficiency
/ diagnosis
Secondary Prevention
/ methods
Stem Cell Transplantation
/ methods
Survival Analysis
Journal
Journal of cardiovascular medicine (Hagerstown, Md.)
ISSN: 1558-2035
Titre abrégé: J Cardiovasc Med (Hagerstown)
Pays: United States
ID NLM: 101259752
Informations de publication
Date de publication:
01 04 2021
01 04 2021
Historique:
entrez:
26
2
2021
pubmed:
27
2
2021
medline:
16
12
2021
Statut:
ppublish
Résumé
The prognosis for patients affected by light-chain cardiac amyloidosis and acquired transthyretin-related (TTR) amyloidosis is poor. Heart transplantation (HTx) could improve prognosis also enabling autologous stem cell transplantation (ASCT) in the first group. A total of 36 patients affected by systemic amyloidosis have been referred to our centre from 2009 to 2019. Of these, nine had cardiac involvement: seven by light-chain amyloidosis and two by acquired TTR amyloidosis. None died while waiting for HTx. A specific internal protocol useful to select candidates and to monitor the organ involvement after HTx was developed. Median age at diagnosis was 54 years and 66% were male. The most common short-term complication after HTx was renal failure (44%), followed by acute cardiac rejection more than 2R (22%). ASCT was performed in six out of seven light-chain cardiac amyloidosis patients, with a median time of 6 months after HTx. Two patients affected by light-chain cardiac amyloidosis died due to amyloidosis relapse: one before undergoing ASCT. After a median follow-up of 31 (7-124) months, 1- and 5-year survival was 88 and 66% in the cardiac light-chain amyloidosis group. Conversely, 1- and 5-year survival was 100% in the acquired TTR amyloidosis group. HTx may represent a valuable option in carefully selected patients. ASCT after HTx is an effective treatment that could decrease amyloidosis relapse in light-chain cardiac amyloidosis patients. A multidisciplinary approach is mandatory to select the best candidates and to obtain the most effective results with a specific surveillance follow-up protocol.
Identifiants
pubmed: 33633041
doi: 10.2459/JCM.0000000000001094
pii: 01244665-202104000-00004
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
261-267Informations de copyright
Copyright © 2020 Italian Federation of Cardiology - I.F.C. All rights reserved.
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