OBJECTIVE: Cardiac amyloidosis (CA) is associated with a poor prognosis and a survival rate of less than 30% 2 years after clinical manifestation. Considered as a semi-malignant disease, CA is often a contraindication for HTx; however, depending on the type of CA, there are excellent treatment regimes that can be combined with HTx. In AL-amyloidosis, chemotherapy and stem cell transplantation are necessary and in TTR-amyloidosis, where the liver is the source of the pathologic protein, liver transplantation is recommended after HTx. METHODS AND RESULTS: More than 60 patients with AL-amyloidosis and more than 25 patients with ATTR-amyloidosis have been investigated at our centre. Eighteen patients showed signs of end-stage heart failure. Four patients died within 1 month after listing for HTx. Seven patients with AL (mean age 41.8 years) and five patients with ATTR-amyloidosis (mean age 42.6 years) were successfully transplanted with an actual survival rate of 91.6%. One patient died 8 months after HTx due to infection. Five AL patients received chemotherapy and SCT and one ATTR patient was liver transplanted. Three AL patients showed complete remission of amyloidosis. CONCLUSIONS: Cardiac amyloidosis is a potentially curative disease after HTx when combined with either chemotherapy and SCT or LiverTx depending on the type of the amyloidosis. Due to the natural course of the disease, urgent HTx after cardiac manifestation is mandatory. With this approach, excellent survival rates and even remission of the underlying disease is possible.
OBJECTIVE:Cardiac amyloidosis (CA) is associated with a poor prognosis and a survival rate of less than 30% 2 years after clinical manifestation. Considered as a semi-malignant disease, CA is often a contraindication for HTx; however, depending on the type of CA, there are excellent treatment regimes that can be combined with HTx. In AL-amyloidosis, chemotherapy and stem cell transplantation are necessary and in TTR-amyloidosis, where the liver is the source of the pathologic protein, liver transplantation is recommended after HTx. METHODS AND RESULTS: More than 60 patients with AL-amyloidosis and more than 25 patients with ATTR-amyloidosis have been investigated at our centre. Eighteen patients showed signs of end-stage heart failure. Four patients died within 1 month after listing for HTx. Seven patients with AL (mean age 41.8 years) and five patients with ATTR-amyloidosis (mean age 42.6 years) were successfully transplanted with an actual survival rate of 91.6%. One patient died 8 months after HTx due to infection. Five ALpatients received chemotherapy and SCT and one ATTRpatient was liver transplanted. Three ALpatients showed complete remission of amyloidosis. CONCLUSIONS:Cardiac amyloidosis is a potentially curative disease after HTx when combined with either chemotherapy and SCT or LiverTx depending on the type of the amyloidosis. Due to the natural course of the disease, urgent HTx after cardiac manifestation is mandatory. With this approach, excellent survival rates and even remission of the underlying disease is possible.
Authors: Barbara Kieninger; Magdalena Eriksson; Reinhard Kandolf; Philipp A Schnabel; Stefan Schönland; Arnt V Kristen; Ute Hegenbart; Peter Lohse; Christoph Röcken Journal: Virchows Arch Date: 2010-04-08 Impact factor: 4.064
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: Ashutosh D Wechalekar; Mark Offer; Julian D Gillmore; Philip N Hawkins; Helen J Lachmann Journal: Nat Clin Pract Cardiovasc Med Date: 2008-12-17
Authors: A V Kristen; S O Schönland; A Remppis; U Hegenbart; P A Schnabel; H A Katus; T J Dengler Journal: Pathologe Date: 2009-05 Impact factor: 1.011
Authors: V Audard; M Matignon; L Weiss; P Remy; A Pardon; C Haioun; K Belhadj; L Salomon; M L Hillon; D Sahali; E Vermes; P Lang; P Grimbert Journal: Am J Transplant Date: 2008-11-27 Impact factor: 8.086
Authors: Sanjay M Banypersad; James C Moon; Carol Whelan; Philip N Hawkins; Ashutosh D Wechalekar Journal: J Am Heart Assoc Date: 2012-04-24 Impact factor: 5.501