Author:
Razvi Yousuf,Porcari Aldostefano,Di Nora Concetta,Patel Rishi K.,Ioannou Adam,Rauf Muhammad U.,Masi Ambra,Law Steven,Chacko Liza,Rezk Tamer,Ravichandran Sriram,Gilbertson Janet,Rowczenio Dorota,Blakeney Iona J.,Kaza Nandita,Hutt David F.,Lachmann Helen,Wechalekar Ashutosh,Moody William,Lim Sern,Chue Colin,Whelan Carol,Venneri Lucia,Martinez-Naharro Ana,Merlo Marco,Sinagra Gianfranco,Livi Ugolino,Hawkins Philip,Fontana Marianna,Gillmore Julian D.
Abstract
AimsTransthyretin cardiac amyloidosis (ATTR-CM) is a progressive and fatal cardiomyopathy. Treatment options in patients with advanced ATTR-CM are limited to cardiac transplantation (CT). Despite case series demonstrating comparable outcomes with CT between patients with ATTR-CM and non-amyloid cardiomyopathies, ATTR-CM is considered to be a contraindication to CT in some centers, partly due to a perceived risk of amyloid recurrence in the allograft. We report long-term outcomes of CT in ATTR-CM at two tertiary centers.Materials and methods and ResultsWe retrospectively evaluated ATTR-CM patients across two tertiary centers who underwent transplantation between 1990 and 2020. Pre-transplantation characteristics were determined and outcomes were compared with a cohort of non-transplanted ATTR-CM patients. Fourteen (12 male, 2 female) patients with ATTR-CM underwent CT including 11 with wild-type ATTR-CM and 3 with variant ATTR-CM (ATTRv). Median age at CT was 62 years and median follow up post-CT was 66 months. One, three, and five-year survival was 100, 92, and 90%, respectively and the longest surviving patient was Censored > 19 years post CT. No patients had recurrence of amyloid in the cardiac allograft. Four patients died, including one with ATTRv-CM from complications of leptomeningeal amyloidosis. Survival among the cohort of patients who underwent CT was significantly prolonged compared to UK patients with ATTR-CM generally (p < 0.001) including those diagnosed under age 65 years (p = 0.008) or with early stage cardiomyopathy (p < 0.001).ConclusionCT is well-tolerated, restores functional capacity and improves prognosis in ATTR-CM. The risk of amyloid recurrence in the cardiac allograft appears to be low.
Subject
Cardiology and Cardiovascular Medicine
Cited by
5 articles.
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