Arnt V Kristen1, Michael M Kreusser2, Patrick Blum1, Stefan O Schönland3, Lutz Frankenstein1, Andreas O Dösch1, Benjamin Knop1, Matthias Helmschrott1, Bastian Schmack4, Arjang Ruhparwar5, Ute Hegenbart3, Hugo A Katus2, Philip W J Raake6. 1. Division of Cardiology, Department of Internal Medicine III, University of Heidelberg, Heidelberg, Germany. 2. Division of Cardiology, Department of Internal Medicine III, University of Heidelberg, Heidelberg, Germany; DZHK (German Centre for Cardiovascular Research), partner site Heidelberg/Mannheim, Heidelberg, Germany. 3. Division of Hematology and Oncology, Department of Internal Medicine V. 4. Department of Cardiac Surgery, University of Heidelberg, Heidelberg, Germany. 5. DZHK (German Centre for Cardiovascular Research), partner site Heidelberg/Mannheim, Heidelberg, Germany; Department of Cardiac Surgery, University of Heidelberg, Heidelberg, Germany. 6. Division of Cardiology, Department of Internal Medicine III, University of Heidelberg, Heidelberg, Germany; DZHK (German Centre for Cardiovascular Research), partner site Heidelberg/Mannheim, Heidelberg, Germany. Electronic address: philip.raake@med.uni-heidelberg.de.
Abstract
BACKGROUND: Cardiac amyloidosis, caused most commonly by deposition of light chain (AL) or transthyretin (ATTR) type fibrils, has an extremely poor prognosis. In this retrospective single-center study, we evaluated temporal trends in survival after heart transplantation for cardiac amyloidosis. METHODS: We analyzed 48 patients with cardiac amyloidosis (AL, n = 32; familial ATTR, n = 16) who underwent heart transplantation from May 2002 to March 2017. Patients were analysed in 2 periods, Era 1 (2002- 2007) and Era 2 (2008- 2017), separated by altered patient selection in both, AL and ATTR amyloidosis, and changed chemotherapy regimens for AL amyloidosis. RESULTS: The modern era was characterized by a lower number of extracardiac organ involvement for AL (94% isolated cardiac amyloidosis in Era 2 vs 56% in Era 1; p = 0.0221), and more frequent treatment for AL with the proteasome inhibitor bortezomib (94% in Era 2 vs 6% in Era 1; p < 0.0001). AL patients had significantly lower survival than patients with non-amyloid cardiomyopathy after heart transplantation in Era 1, and ATTR patients had numerically lower survival. However, survival in the modern era was comparable to non-amyloid transplants in both cohorts, possibly reflecting a shift in chemotherapy strategies and patient selection, respectively. CONCLUSIONS: In the current era, use of enhanced chemotherapy regimens for isolated advanced AL cardiac amyloidosis was associated with outcomes comparable to non-amyloid cardiomyopathy. We conclude that heart transplantation in highly selected patients with isolated non-systemic advanced cardiac amyloidosis may be a feasible approach.
BACKGROUND:Cardiac amyloidosis, caused most commonly by deposition of light chain (AL) or transthyretin (ATTR) type fibrils, has an extremely poor prognosis. In this retrospective single-center study, we evaluated temporal trends in survival after heart transplantation for cardiac amyloidosis. METHODS: We analyzed 48 patients with cardiac amyloidosis (AL, n = 32; familial ATTR, n = 16) who underwent heart transplantation from May 2002 to March 2017. Patients were analysed in 2 periods, Era 1 (2002- 2007) and Era 2 (2008- 2017), separated by altered patient selection in both, AL and ATTRamyloidosis, and changed chemotherapy regimens for AL amyloidosis. RESULTS: The modern era was characterized by a lower number of extracardiac organ involvement for AL (94% isolated cardiac amyloidosis in Era 2 vs 56% in Era 1; p = 0.0221), and more frequent treatment for AL with the proteasome inhibitor bortezomib (94% in Era 2 vs 6% in Era 1; p < 0.0001). AL patients had significantly lower survival than patients with non-amyloid cardiomyopathy after heart transplantation in Era 1, and ATTRpatients had numerically lower survival. However, survival in the modern era was comparable to non-amyloid transplants in both cohorts, possibly reflecting a shift in chemotherapy strategies and patient selection, respectively. CONCLUSIONS: In the current era, use of enhanced chemotherapy regimens for isolated advanced AL cardiac amyloidosis was associated with outcomes comparable to non-amyloid cardiomyopathy. We conclude that heart transplantation in highly selected patients with isolated non-systemic advanced cardiac amyloidosis may be a feasible approach.
Authors: Jan M Griffin; Ersilia M DeFilippis; Hannah Rosenblum; Veli K Topkara; Justin A Fried; Nir Uriel; Koji Takeda; Maryjane A Farr; Mathew S Maurer; Kevin J Clerkin Journal: Clin Transplant Date: 2020-10-28 Impact factor: 2.863
Authors: Marcus V Simões; Fabio Fernandes; Fabiana G Marcondes-Braga; Philip Scheinberg; Edileide de Barros Correia; Luis Eduardo P Rohde; Fernando Bacal; Silvia Marinho Martins Alves; Sandrigo Mangini; Andréia Biolo; Luis Beck-da-Silva; Roberta Shcolnik Szor; Wilson Marques Junior; Acary Souza Bulle Oliveira; Márcia Waddington Cruz; Bruno Vaz Kerges Bueno; Ludhmila Abrahão Hajjar; Aurora Felice Castro Issa; Felix José Alvarez Ramires; Otavio Rizzi Coelho Filho; André Schmidt; Ibraim Masciarelli Francisco Pinto; Carlos Eduardo Rochitte; Marcelo Luiz Campos Vieira; Cláudio Tinoco Mesquita; Celso Dario Ramos; José Soares-Junior; Minna Moreira Dias Romano; Wilson Mathias Junior; Marcelo Iório Garcia Junior; Marcelo Westerlund Montera; Marcelo Dantas Tavares de Melo; Sandra Marques E Silva; Pedro Manoel Marques Garibaldi; Aristóteles Comte de Alencar Neto; Renato Delascio Lopes; Diane Xavier de Ávila; Denizar Viana; José Francisco Kerr Saraiva; Manoel Fernandes Canesin; Glaucia Maria Moraes de Oliveira; Evandro Tinoco Mesquita Journal: Arq Bras Cardiol Date: 2021-09 Impact factor: 2.000
Authors: Jan M Griffin; Leonard Chiu; Kelly M Axsom; Rachel Bijou; Kevin J Clerkin; Paolo Colombo; Margaret O Cuomo; Jeffeny De Los Santos; Justin A Fried; Jeff Goldsmith; Marlena Habal; Jennifer Haythe; Stephen Helmke; Evelyn M Horn; Farhana Latif; Sun Hi Lee; Edward F Lin; Yoshifumi Naka; Jayant Raikhelkar; Susan Restaino; Gabriel T Sayer; Hiroo Takayama; Koji Takeda; Sergio Teruya; Veli Topkara; Emily J Tsai; Nir Uriel; Melana Yuzefpolskaya; Maryjane A Farr; Mathew S Maurer Journal: Clin Transplant Date: 2020-07-24 Impact factor: 2.863