Cardiac transplantation in transthyretin amyloid cardiomyopathy: Outcomes from three decades of tertiary center experience
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 card...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2023-01-01
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| Series: | Frontiers in Cardiovascular Medicine |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fcvm.2022.1075806/full |
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| author | Yousuf Razvi Aldostefano Porcari Aldostefano Porcari Concetta Di Nora Rishi K. Patel Adam Ioannou Muhammad U. Rauf Ambra Masi Steven Law Liza Chacko Tamer Rezk Sriram Ravichandran Janet Gilbertson Dorota Rowczenio Iona J. Blakeney Nandita Kaza David F. Hutt Helen Lachmann Ashutosh Wechalekar William Moody Sern Lim Colin Chue Carol Whelan Lucia Venneri Ana Martinez-Naharro Marco Merlo Gianfranco Sinagra Ugolino Livi Philip Hawkins Marianna Fontana Julian D. Gillmore |
| author_facet | Yousuf Razvi Aldostefano Porcari Aldostefano Porcari Concetta Di Nora Rishi K. Patel Adam Ioannou Muhammad U. Rauf Ambra Masi Steven Law Liza Chacko Tamer Rezk Sriram Ravichandran Janet Gilbertson Dorota Rowczenio Iona J. Blakeney Nandita Kaza David F. Hutt Helen Lachmann Ashutosh Wechalekar William Moody Sern Lim Colin Chue Carol Whelan Lucia Venneri Ana Martinez-Naharro Marco Merlo Gianfranco Sinagra Ugolino Livi Philip Hawkins Marianna Fontana Julian D. Gillmore |
| author_sort | Yousuf Razvi |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-10T21:38:35Z |
| format | Article |
| id | doaj.art-dcd42138c72146719916644456120435 |
| institution | Directory Open Access Journal |
| issn | 2297-055X |
| language | English |
| last_indexed | 2024-04-10T21:38:35Z |
| publishDate | 2023-01-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Cardiovascular Medicine |
| spelling | doaj.art-dcd42138c721467199166444561204352023-01-19T07:47:14ZengFrontiers Media S.A.Frontiers in Cardiovascular Medicine2297-055X2023-01-01910.3389/fcvm.2022.10758061075806Cardiac transplantation in transthyretin amyloid cardiomyopathy: Outcomes from three decades of tertiary center experienceYousuf Razvi0Aldostefano Porcari1Aldostefano Porcari2Concetta Di Nora3Rishi K. Patel4Adam Ioannou5Muhammad U. Rauf6Ambra Masi7Steven Law8Liza Chacko9Tamer Rezk10Sriram Ravichandran11Janet Gilbertson12Dorota Rowczenio13Iona J. Blakeney14Nandita Kaza15David F. Hutt16Helen Lachmann17Ashutosh Wechalekar18William Moody19Sern Lim20Colin Chue21Carol Whelan22Lucia Venneri23Ana Martinez-Naharro24Marco Merlo25Gianfranco Sinagra26Ugolino Livi27Philip Hawkins28Marianna Fontana29Julian D. Gillmore30Division of Medicine, National Amyloidosis Centre, Royal Free Hospital, University College London, London, United KingdomDivision of Medicine, National Amyloidosis Centre, Royal Free Hospital, University College London, London, United KingdomCardiovascular Department, Centre for Diagnosis and Treatment of Cardiomyopathies, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI), University of Trieste, Trieste, ItalyDepartment of Cardiothoracic Science, Azienda Sanitaria Universitaria Integrata di Udine, Udine, ItalyDivision of Medicine, National Amyloidosis Centre, Royal Free Hospital, University College London, London, United KingdomDivision of Medicine, National Amyloidosis Centre, Royal Free Hospital, University College London, London, United KingdomDivision of Medicine, National Amyloidosis Centre, Royal Free Hospital, University College London, London, United KingdomDivision of Medicine, National Amyloidosis Centre, Royal Free Hospital, University College London, London, United KingdomDivision of Medicine, National Amyloidosis Centre, Royal Free Hospital, University College London, London, United KingdomDivision of Medicine, National Amyloidosis Centre, Royal Free Hospital, University College London, London, United KingdomDivision of Medicine, National Amyloidosis Centre, Royal Free Hospital, University College London, London, United KingdomDivision of Medicine, National Amyloidosis Centre, Royal Free Hospital, University College London, London, United KingdomDivision of Medicine, National Amyloidosis Centre, Royal Free Hospital, University College London, London, United KingdomDivision of Medicine, National Amyloidosis Centre, Royal Free Hospital, University College London, London, United KingdomDivision of Medicine, National Amyloidosis Centre, Royal Free Hospital, University College London, London, United KingdomImperial College London, London, United KingdomDivision of Medicine, National Amyloidosis Centre, Royal Free Hospital, University College London, London, United KingdomDivision of Medicine, National Amyloidosis Centre, Royal Free Hospital, University College London, London, United KingdomDivision of Medicine, National Amyloidosis Centre, Royal Free Hospital, University College London, London, United KingdomDepartment of Cardiology, Queen Elizabeth Hospital Birmingham, Birmingham, United KingdomDepartment of Cardiology, Queen Elizabeth Hospital Birmingham, Birmingham, United KingdomDepartment of Cardiology, Queen Elizabeth Hospital Birmingham, Birmingham, United KingdomDivision of Medicine, National Amyloidosis Centre, Royal Free Hospital, University College London, London, United KingdomDivision of Medicine, National Amyloidosis Centre, Royal Free Hospital, University College London, London, United KingdomDivision of Medicine, National Amyloidosis Centre, Royal Free Hospital, University College London, London, United KingdomCardiovascular Department, Centre for Diagnosis and Treatment of Cardiomyopathies, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI), University of Trieste, Trieste, ItalyCardiovascular Department, Centre for Diagnosis and Treatment of Cardiomyopathies, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI), University of Trieste, Trieste, ItalyDepartment of Cardiothoracic Science, Azienda Sanitaria Universitaria Integrata di Udine, Udine, ItalyDivision of Medicine, National Amyloidosis Centre, Royal Free Hospital, University College London, London, United KingdomDivision of Medicine, National Amyloidosis Centre, Royal Free Hospital, University College London, London, United KingdomDivision of Medicine, National Amyloidosis Centre, Royal Free Hospital, University College London, London, United KingdomAimsTransthyretin 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.https://www.frontiersin.org/articles/10.3389/fcvm.2022.1075806/fullamyloidtransplantheart failureTTR–transthyretinoutcome |
| spellingShingle | Yousuf Razvi Aldostefano Porcari Aldostefano Porcari Concetta Di Nora Rishi K. Patel Adam Ioannou Muhammad U. Rauf Ambra Masi Steven Law Liza Chacko Tamer Rezk Sriram Ravichandran Janet Gilbertson Dorota Rowczenio Iona J. Blakeney Nandita Kaza David F. Hutt Helen Lachmann Ashutosh Wechalekar William Moody Sern Lim Colin Chue Carol Whelan Lucia Venneri Ana Martinez-Naharro Marco Merlo Gianfranco Sinagra Ugolino Livi Philip Hawkins Marianna Fontana Julian D. Gillmore Cardiac transplantation in transthyretin amyloid cardiomyopathy: Outcomes from three decades of tertiary center experience Frontiers in Cardiovascular Medicine amyloid transplant heart failure TTR–transthyretin outcome |
| title | Cardiac transplantation in transthyretin amyloid cardiomyopathy: Outcomes from three decades of tertiary center experience |
| title_full | Cardiac transplantation in transthyretin amyloid cardiomyopathy: Outcomes from three decades of tertiary center experience |
| title_fullStr | Cardiac transplantation in transthyretin amyloid cardiomyopathy: Outcomes from three decades of tertiary center experience |
| title_full_unstemmed | Cardiac transplantation in transthyretin amyloid cardiomyopathy: Outcomes from three decades of tertiary center experience |
| title_short | Cardiac transplantation in transthyretin amyloid cardiomyopathy: Outcomes from three decades of tertiary center experience |
| title_sort | cardiac transplantation in transthyretin amyloid cardiomyopathy outcomes from three decades of tertiary center experience |
| topic | amyloid transplant heart failure TTR–transthyretin outcome |
| url | https://www.frontiersin.org/articles/10.3389/fcvm.2022.1075806/full |
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