| Přispěvatelé: |
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 |
| Popis: |
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. |
