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2. Diagnosing AL and ATTR Amyloid Cardiomyopathy: A Multidisciplinary Approach.

Author

aus dem Siepen, Fabian and Hansen, Timon

Subjects
*HEART failure, *CARDIAC amyloidosis, *AMYLOIDOSIS, *CARDIOMYOPATHIES, *TRANSTHYRETIN, *EARLY diagnosis
Abstract

Amyloidosis with cardiac involvement is a fatal disease leading to progressive heart failure. The most common forms of amyloidosis with cardiac involvement are light chain (AL) and transthyretin (ATTR) amyloidosis. To allow effective specific treatment for both forms, precise and early diagnosis is important. This review focuses on diagnostic approaches for AL and ATTR amyloidosis with cardiac involvement, including different strategies, the role of imaging and biomarkers and possible pitfalls. [ABSTRACT FROM AUTHOR]

Published
2024
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3. Response to therapy with tafamidis 61 mg in patients with cardiac transthyretin amyloidosis: real-world experience since approval.

Author

aus dem Siepen, Fabian, Meissner, Christopher, Hofmann, Eva, Hein, Selina, Nagel, Christian, Hegenbart, Ute, Schönland, Stefan O., Andre, Florian, Frey, Norbert, and Kristen, Arnt V.

Subjects
*BRAIN natriuretic factor, *SURVIVAL rate, *KARNOFSKY Performance Status, *VENTRICULAR ejection fraction, *GLOMERULAR filtration rate, *CARDIAC amyloidosis, *HEART failure
Abstract

Aims: Transthyretin amyloid cardiomyopathy (ATTR-CM) is a progressive disease that causes heart failure due to amyloid fibril deposition. Tafamidis was approved as the first causal treatment in 2020. We here report on real-world data in patients treated with tafamidis for at least 12 months according to the recently defined European Society for Cardiology (ESC) consensus criteria for disease progression. Methods and results: Three hundred and eight wildtype and 31 hereditary ATTR-CM patients were prospectively enrolled after first diagnosis of ATTR-CM and initiation of tafamidis 61 mg once daily treatment. After 12 months, significant deterioration in Karnofsky Index, estimated glomerular filtration rate (eGFR), N-terminal brain natriuretic peptide (NT-proBNP), septum thickness and left ventricular ejection fraction (LVEF) could be observed, significant disease progression was only detected in 25 patients (9%) using ESC consensus criteria. Mean survival time was 37 months with no differences between responders and non-responders. NT-proBNP was the only independent predictor for poor therapy response (p =.008). Conclusions: The majority of patients showed no significant disease progression according to the ESC consensus criteria after 12 months of therapy with tafamidis. However, at 12 months, treatment response based on the ESC consensus criteria was not associated with improved survival. Moreover, higher levels of NT-proBNP at diagnosis of ATTR-CM appears to predict poorer treatment response, confirming that timely initiation of therapy is advantageous. [ABSTRACT FROM AUTHOR]

Published
2024
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5. Long-term safety and efficacy of patisiran for hereditary transthyretin-mediated amyloidosis with polyneuropathy: 12-month results of an open-label extension study

Author

Vita, Giuseppe, Rizzo, Vincenzo, Russo, Massimo, Mazzeo, Anna, Gentile, Luca, Berk, John L, Brueckner, Caitlin, Lazzari, Victoria, Wiesman, Janice, DeLong, Douglas, Victory, Jennifer, Dalton, James, May, John, Gilmore, Catherine, Attarian, Shahram, Diallo, Saran, Delmont, Emilien, Pouget, Jean, Verschueren, Annie, Grapperon, Aude-Marie, Campana-Salort, Emmanuelle, Conceição, Isabel M, Lopes, Ana, Lamas, Filipa, Neves, Carlos, Castro, Jose, Pereira, Pedro, Castro, Isabel, Franco, Ana, Santos, Miguel Oliveira, de Azevedo Coutinho, Conceição, Falcao de Campos, Catarina, Coelho, Teresa, Hipólito Reis, Antonio, Correia, Nuno, Perez, Javier M, Martins da Silva, Ana, Alves, Cristina, Cardoso, Marcio, Valdrez, Katia, Monte, Julia R, Pessoa, Bernardete, Guimaraes, Nadia, Freitas, Monica, Ramalho, Joana, Ferreira, Natalia, Kuzume, Daisuke, Tard, Celine, Waucquier, Nawal, Rougeaux, Isabelle, Brice, Sylvie, Kasprzyk, Emmanuelle, Elrezzi, Elise, Meguig, Sayah, Hachulla, Eric, Gauvain, Clement, Migaud-Chervy, Maria-Claire, Deplanque, Dominique, Jozefowicz, Elsa, Lebellec, Loic, Adams, David, Balaya-Gouraya, Line, Jehan Lacour, Nathalie, Bournane, Halima, Martin, Nathalie, Elabed, Mongia, Sacko, Niamey, Boubrit, Yasmine, Gaouar, Amina, Rakotondratafika, Fetra, Théaudin-Saliou, Marie, Cauquil-Michon, Cécile, Labeyrie, Celine, Not, Adeline, Al-Salameh, Abdallah, Lecoq, Anne-Lise, Stephant, Maeva, Echaniz-Laguna, Andoni, Becquemont, Laurent, Beaudonnet, Guillemette, Algalarrondo, Vincent, Eliahou, Ludivine, Slama, Michel S, Rousseau, Antoine, Signate, Aissatou, Berthelot, Emeline, Inamo, Jocelyn, Planté-Bordeneuve, Violaine, Vervoitte, Laetitia, Focseneanu, Cecile, Gendre, Thierry, Arrouasse, Raphaele, Ayache, Samar S., Ernande, Laura, Le Corvoisier, Philippe, Salhi, Hayet, Choumert, Ariane, Ehinger, Vincent, Ruiz, Julie, Charlin, Cyril, Megelin, Thomas, Brannagan III, Thomas H, Fayerman, Raisy, Kim, Arreum, Paras, Allan, Gonzalez, Leidy J, Tsang, Steven, Wajnsztajn, Fernanda, Shije, Jeffrey, Ulane, Christina, Kleyman, Inna, Weimer, Louis, Cioroiu, Comana, Lambrianides, Sakis, Abu-Manneh, Rana, Zamba-Papanicolaou, Eleni, Agathangelou, Petros, Leonidou, Eleni, Tada, Satoshi, Fujita, Akemi, Nagai, Masahiro, Ando, Rina, Hosokawa, Yuko, Yamanishi, Yuki, Overcash, J. Scott, Giardino, Elena, Boyer, Leslie, Dang, Lien, Le, An, Nguyen, Tyler, Giang, Lien, Sellers, Peter, Tran, Leyla, Truong, Nghi, Vinas, Maita, Hrkman, Nicole, Miller, Sarah, Nguyen, David, Smith, Ashley, Pu, Helen, Li, Steve, Vuong, Thao, Dioso, Holly, Green, Sinikka, Lee, Kia, Chu, Hanh, Waters, Michael, Coskun, Derya J, Zepeda, Karla A, O'Riordan, William, Obici, Laura, Cortese, Andrea, Lozza, Alessandro, Merlini, Giampaolo, Rosti, Vittorio, Sabatelli, Mario, Bisogni, Giulia, Bernardo, Daniela, Luigetti, Marco, Di Paolantonio, Andrea, Guglielmino, Valeria, Romano, Angela, Nienhuis, Hans, Bulthuis-Kuiper, Janita, Kristen, Arnt V, Gerk, Olga, Ulbricht, Hannah, Taylor, Lenka, Meyle, Eva, Kleinschmidt, Natalia, Meyrath, David, Noe-Schwenn, Simone, Meng, Ulrike, Bauer, Ralf, aus dem Siepen, Fabian, Hein, Selina, Takahashi, Tetsuya, Oshita, Tomohiko, Koujin, Yoko, Neshige, Shuichiro, Nezu, Tomohisa, Segawa, Akiko, Ueno, Hiroki, Morino, Hiroyuki, Campistol, Josep M, Rodas Marin, Lida Maria, Blasco Pelicano, Josep Miquel, Dávila, Lucía Galán, Palacios, Marta, Pytel Cordoba, Vanesa, Guerrero Sola, Antonio, Horga, Alejandro, García Feijoo, Julián, Perez de Isla, Leopoldo, Marques Júnior, Wilson, Moscardini, Mariana, Litcanov, Debora Cristina, Viera Lima, Ana Flavia, Rodrigues, Leonardo, Marques Coutinho, Barbara, Moreira, Carolina Lavigne, Daccach Marques, Vanessa, Munoz Beamud, Francisco, Gragera Martínez, Álvaro, Borrachero, Cristina, Losada López, Inés Asunción, Cisneros Barroso, Eugenia, Rodríguez Rodríguez, Adrián, Sanz, Monica, Rigo Oliver, Elena, González Moreno, Juan, Gamez Martinez, Jose M, Descals, Cristina, Uson, Mercedes, Jose Vega, Francisco, Figuerola, Antoni, Montala, Carles, Waddington-Cruz, Márcia, Dias da Silva, Moises, Gervais de Santa Rosa, Renata, Pinto, Luiz Felipe, Pinto, Marcus Vinicius, Cardoso Berensztejn, Amanda, Barroso, Fabio, Lautre, Andrea, Orellana, Lucas G, González-Duarte Briseño, Maria Alejandra, Cárdenas-Soto, Karla, Jiménez López, Brenda Poled, Pérez-Castañeda, Sandra Lorena, Cantú Brito, Carlos Gerardo, Rivera de la Parra, David, Hernandez Reyes, Jose Pablo, del Mar Saniger Alba, Maria, Criollo Mora, Elia, Parman, Yesim, Rezzan, Kus Jülide, Sahin, Erdi, Serbest, Nail G, Durmus, Hacer, Cakar, Arman, Tugal Tutkun, Nuriye Ilknur, Karamursel, Sacit, Elitok, Ali, Sirin Inan, Nermin G, Altinkurt, Emre, Polydefkis, Michael, Ye, Jing, Allen, Adriane C, Chaudhry, Vinay, Jarrett, Raquel, Bressler, Neil, Burks, Kathleen L, Liu, Qingfeng, Khoshnoodi, Mohammad, Judge, Daniel P, Vista, Geno, Shah, Syed Mahmood, Hamaguchi, Hirotoshi, Oda, Junko, Fukase, Emi, Taniguchi, Ikuko, Oda, Tetsuya, Endo, Hironobu, Shimomura, Masahiro, Katanazaka, Kimitaka, Koto, Shusuke, Nakano, Takahiro, Scheid, Christof, Zueiter, Andreas, Pester, Lars, Walter, Doreen, Özdemir, Betül, Frenzel, Lukas F, Holtick, Udo, Oh, Jeeyoung, Kim, Hee Jin, Shin, Hyun Jin, Choi, Kyomin, Yamashita, Taro, Ueda, Mitsuharu, Masuda, Teruaki, Misumi, Yohei, Ueda, Akihiko, Nakahara, Keiichi, Yorita, Akiko, Tsuruhisa, Seiko, Taniwaki, Takayuki, Harada, Masaya, Moritaka, Taiga, Sakurada, Naonori, Mauricio, Elizabeth A, Baskin, Amber, Dimberg, Elliot, Dispenzieri, Angela, Fonder, Amie, Hobbs, Miriam, Russell, Stephen J, Dyck, Peter, Gonsalves, Wilson, Leung, Nelson, Witzig, Thomas E, Zeldenrust, Steven R, Hwa, Lisa, Kapoor, Prashant, Kumar, Shaji K, Lin, Yi, Lust, John A, Rajkumar, Vincent S, Dingli, David, Gertz, Morie A, Go, Ronald, Hayman, Suzanne R, Dalia, Samir, Carrillo, Esmeralda, Gorevic, Peter, Mason, Garnette, Chao, Chi-Chao, Lee, Ming-Jen, Su, Jen-Jen, Hsieh, Sung-Tsang, Tsai, Li-Kai, Yeh, Shin-Joe, Yang, Chih-Chao, Ajroud-Driss, Senda Ajroud-Driss, Casey, Patricia, Joslin, Benjamin C, Freimer, Miriam, Sankey, Alison, Kenepp, Amanda, Heintzman, Sarah, LoRusso, Samantha, Hokezu, Youichi, Kim, Byoung-Joon, Kim, JuHyeon, Lee, Ga Yeon, Cho, Eun Bin, Jeon, Eun-Seok, Min, Ju-Hong, Seok, Jin Myoung, Lee, Hye Lim, Park, Jae Hong, Sekijima, Yoshiki, Miyazawa, Chinatsu, Kato, Nagaaki, Kishida, Dai, Hineno, Akiyo, Kodaira, Minori, Yoshinaga, Tsuneaki, Miyahara, Teruyoshi, Imai, Akira, Matsumoto, Kazuhiko, Lin, Kon-Ping, Lee, Yi-Chung, Wixner, Jonas, Falk, Malin, Pilebro, Bjorn, Suhr, Ole, Lindqvist, Per, Soderberg, Karin, Pedrosa-Domellöf, Fatima, Anan, Intissar, Nordh, Erik, Tournev, Ivaylo, Zhelyazkova-Glaveeva, Sashka, Cherneva, Zheyna, Sarafov, Staiko, Chamova, Teodora, Cherninkova-Gopina, Sylvia, Schmidt, Hartmut H, Friebel, Frauke, Zibert, Andree, Mihailovic, Natasa, Schubert, Friederike, Vorona, Elena, Lahme, Larissa, Huesing-Kabar, Anna, Schilling, Matthias, Kabar, Iyad, Gillmore, Julian D, Martinez-Naharro, Ana, Chacko, Liza, Cohen, Oliver, Law, Steven, Rezk, Tamer, Lachmann, Helen J, Quan, Dianna, Blume, Brianna, Dixon, Stacy, Low, Soon Chai, Chan, Soo Looi, Lim, He Eng Li, Goh, Khean Jin, Mezei, Michelle M, Kraus, Deborah, Jack, Kristin, Wade, N. Kevin, Lopate, Glenn, Zwijack, Brittany, Florence, Julaine, Sommerville, R. Brian, Stewart, Graeme, Ryder, Julie, Mekhael, Linda, Taylor, Mark, Suan, Daniel, Wells, Karen, Stone, Paula, Itoya, Amenze, Owusu-Sekyere, Mercy, Thai, Desmond, Chahine, Ilonah, Pedrosa, Salve, Do, Thi Hoa (Therese), González-Duarte, Alejandra, Kyriakides, Theodoros, Ajroud-Driss, Senda, Mauricio, Elizabeth, Brannagan, Thomas H, III, Aldinc, Emre, Wang, Jing Jing, White, Matthew T, Vest, John, Berber, Erhan, and Sweetser, Marianne T

Published
2021
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8. Prognosis of light chain amyloidosis: a multivariable analysis for survival prediction in patients with cardiac involvement proven by endomyocardial biopsy

Author

Aurich, Matthias, primary, Bucur, Julian, additional, Vey, Johannes A, additional, Greiner, Sebastian, additional, aus dem Siepen, Fabian, additional, Hegenbart, Ute, additional, Schönland, Stefan, additional, Katus, Hugo A, additional, Frey, Norbert, additional, and Mereles, Derliz, additional

Published
2023
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9. Z-score mapping for standardized analysis and reporting of cardiovascular magnetic resonance modified Look-Locker inversion recovery (MOLLI) T1 data: Normal behavior and validation in patients with amyloidosis

Author

Kranzusch, Riccardo, aus dem Siepen, Fabian, Wiesemann, Stephanie, Zange, Leonora, Jeuthe, Sarah, Ferreira da Silva, Tiago, Kuehne, Titus, Pieske, Burkert, Tillmanns, Christoph, Friedrich, Matthias G., Schulz-Menger, Jeanette, and Messroghli, Daniel R.

Published
2020
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10. Phase 1 Trial of Antibody NI006 for Depletion of Cardiac Transthyretin Amyloid

Author

Garcia-Pavia, Pablo, primary, aus dem Siepen, Fabian, additional, Donal, Erwan, additional, Lairez, Olivier, additional, van der Meer, Peter, additional, Kristen, Arnt V., additional, Mercuri, Michele F., additional, Michalon, Aubin, additional, Frost, Robert J.A., additional, Grimm, Jan, additional, Nitsch, Roger M., additional, Hock, Christoph, additional, Kahr, Peter C., additional, and Damy, Thibaud, additional

Published
2023
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11. Phase 1 Trial of Antibody NI006 for Depletion of Cardiac Transthyretin Amyloid

Author

Garcia-Pavia, Pablo, Aus dem Siepen, Fabian, Donal, Erwan, Lairez, Olivier, van der Meer, Peter, Kristen, Arnt V, Mercuri, Michele F, Michalon, Aubin; https://orcid.org/0000-0002-5246-4881, Frost, Robert J A, Grimm, Jan, Nitsch, Roger M, Hock, Christoph, Kahr, Peter C, Damy, Thibaud, Garcia-Pavia, Pablo, Aus dem Siepen, Fabian, Donal, Erwan, Lairez, Olivier, van der Meer, Peter, Kristen, Arnt V, Mercuri, Michele F, Michalon, Aubin; https://orcid.org/0000-0002-5246-4881, Frost, Robert J A, Grimm, Jan, Nitsch, Roger M, Hock, Christoph, Kahr, Peter C, and Damy, Thibaud

Abstract

BACKGROUND Transthyretin amyloid (ATTR) cardiomyopathy is a progressive and fatal disease caused by misfolded transthyretin. Despite advances in slowing disease progression, there is no available treatment that depletes ATTR from the heart for the amelioration of cardiac dysfunction. NI006 is a recombinant human anti-ATTR antibody that was developed for the removal of ATTR by phagocytic immune cells. METHODS In this phase 1, double-blind trial, we randomly assigned (in a 2:1 ratio) 40 patients with wild-type or variant ATTR cardiomyopathy and chronic heart failure to receive intravenous infusions of either NI006 or placebo every 4 weeks for 4 months. Patients were sequentially enrolled in six cohorts that received ascending doses (ranging from 0.3 to 60 mg per kilogram of body weight). After four infusions, patients were enrolled in an open-label extension phase in which they received eight infusions of NI006 with stepwise increases in the dose. The safety and pharmacokinetic profiles of NI006 were assessed, and cardiac imaging studies were performed. RESULTS The use of NI006 was associated with no apparent drug-related serious adverse events. The pharmacokinetic profile of NI006 was consistent with that of an IgG antibody, and no antidrug antibodies were detected. At doses of at least 10 mg per kilogram, cardiac tracer uptake on scintigraphy and extracellular volume on cardiac magnetic resonance imaging, both of which are imaging-based surrogate markers of cardiac amyloid load, appeared to be reduced over a period of 12 months. The median N-terminal pro-B-type natriuretic peptide and troponin T levels also seemed to be reduced. CONCLUSIONS In this phase 1 trial of the recombinant human antibody NI006 for the treatment of patients with ATTR cardiomyopathy and heart failure, the use of NI006 was associated with no apparent drug-related serious adverse events. (Funded by Neurimmune; NI006-101 ClinicalTrials.gov number, NCT04360434.).

Published
2023

15. Myocardial contraction fraction derived from cardiovascular magnetic resonance cine images—reference values and performance in patients with heart failure and left ventricular hypertrophy

Author

Arenja, Nisha, Fritz, Thomas, Andre, Florian, Riffel, Johannes H, aus dem Siepen, Fabian, Ochs, Marco, Paffhausen, Judith, Hegenbart, Ute, Schönland, Stefan, Müller-Hennessen, Matthias, Giannitsis, Evangelos, Kristen, Arnt V, Katus, Hugo A, Friedrich, Matthias G, and Buss, Sebastian J

Published
2017
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16. Initial experience with transcatheter tricuspid valve repair in patients with cardiac amyloidosis.

Author

Hoerbrand, Isabel A., Volz, Martin J., Aus dem Siepen, Fabian, Aurich, Matthias, Schlegel, Philipp, Geis, Nicolas A., Hegenbart, Ute, Konstandin, Mathias H., Frey, Norbert, and Raake, Philip W.

Subjects
TRICUSPID valve insufficiency, TRICUSPID valve, CARDIAC amyloidosis, TRICUSPID valve surgery, CARDIAC patients, HEART failure patients, TRANSTHYRETIN
Abstract

Aims: Wildtype transthyretin amyloid cardiomyopathy is an under‐recognized cause of heart failure in elderly patients. Transcatheter tricuspid valve repair is a newly emerging therapeutic option for severe tricuspid regurgitation (TR). We present first insights into safety and possible benefits of this procedure in patients with cardiac amyloidosis. Methods and results: Eight patients with cardiac non‐hereditary (wildtype) transthyretin (ATTRwt) amyloidosis and severe to torrential TR, undergoing successful transcatheter tricuspid valve repair, were included in the analysis and compared to a control group of 21 patients without cardiac amyloidosis. All patients presented with an advanced stage of amyloid cardiomyopathy. Primary endpoint was reduction in TR at 3 months follow‐up. Secondary endpoints were feasibility, safety, hospitalization or death, clinical improvement, cardiac biomarkers, and structural and functional right heart parameter obtained by echocardiography. Transcatheter tricuspid valve repair resulted in a significant reduction of TR (IV to II, P = 0.008) in all eight patients with cardiac amyloidosis (100%). Device success (amyloidosis 75% vs. control group 86%, P = 0.597) and overall probability of hospitalization or death (amyloidosis 13% vs. control group 25%, P = 0.646) were similar compared with those in the control group at 3 months follow‐up. Transcatheter tricuspid valve repair led to an improvement of New York Heart Association functional class (P = 0.031) and 6 min walking distance (from 313 ± 118 to 337 ± 106, P = 0.012). TR reduction in amyloidosis patients was less extensive compared with that in control group (TR‐reduction 1.6 ± 0.3, P = 0.008 vs. control group 2.3 ± 0.3, P < 0.0001). Furthermore, these patients showed no significant improvement of structural right heart parameters. Conclusions: Transcatheter tricuspid valve repair is a safe and feasible new treatment option in patients with amyloid cardiomyopathy and has the potential to improve TR‐grade and clinical status. However, the benefit appears to be less pronounced compared with patients without cardiac amyloidosis. [ABSTRACT FROM AUTHOR]

Published
2023
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18. Diagnostic Work-Up of Cardiac Amyloidosis Using Cardiovascular Imaging: Current Standards and Practical Algorithms

Author

Korosoglou,Grigorios, Giusca,Sorin, André,Florian, aus dem Siepen,Fabian, Nunninger,Peter, Kristen,Arnt V, and Frey,Norbert

Subjects
Vascular Health and Risk Management
Abstract

Grigorios Korosoglou,1,2 Sorin Giusca,1,2 Florian André,3,4 Fabian aus dem Siepen,3,4 Peter Nunninger,5 Arnt V Kristen,3,6 Norbert Frey3,4 1GRN Hospital Weinheim, Department of Cardiology, Vascular Medicine and Pneumology, Weinheim, Germany; 2Cardiac Imaging Center Weinheim, Hector Foundation, Weinheim, Germany; 3Department of Cardiology, Pneumology and Angiology, University Hospital Heidelberg, Heidelberg, Germany; 4German Centre for Cardiovascular Research (DZHK), Partner Site Heidelberg, Heidelberg, Germany; 5Radiology and Nuclear Medicine Weinheim, Weinheim, Germany; 6Cardiovascular Center Darmstadt, Darmstadt, GermanyCorrespondence: Grigorios KorosoglouGRN Hospital Weinheim, Department of Cardiology & Vascular Medicine, Roentgenstrasse 1, Weinheim, D-69469, GermanyTel +49 6201 89 2142Fax +49 6201 89 2507Email gkorosoglou@hotmail.comAbstract: Among non-ischemic cardiomyopathies, cardiac amyloidosis is one of the most common, being caused by extracellular depositions of amyloid fibrils in the myocardium. Two main forms of cardiac amyloidosis are known so far, including 1) light-chain (AL) amyloidosis caused by monoclonal production of light-chains, and 2) transthyretin (ATTR) amyloidosis, caused by dissociation of the transthyretin tetramer into monomers. Both AL and ATTR amyloidosis are progressive diseases with median survival from diagnosis of less than 6 months and 3 to 5 years, respectively, if untreated. In this regard, death occurs in most patients due to cardiac causes, mainly congestive heart failure, which can be prevented due to the presence of effective, life-saving treatment regimens. Therefore, early diagnosis of cardiac amyloidosis is crucial more than ever. However, diagnosis of cardiac amyloidosis may be challenging due to variable clinical manifestations and the perceived rarity of the disease. In this regard, clinical and laboratory reg flags are available, which may help clinicians to raise suspicion of cardiac amyloidosis. In addition, advances in cardiovascular imaging have already revealed a higher prevalence of cardiac amyloidosis in specific populations, so that the diagnosis especially of ATTR amyloidosis has experienced a > 30-fold increase during the past ten years. The goal of our review article is to summarize these findings and provide a practical approach for clinicians on how to use cardiovascular imaging techniques, such as echocardiography, cardiac magnetic resonance, bone scintigraphy and, if required, organ biopsy within predefined diagnostic algorithms for the diagnostic work-up of patients with suspected cardiac amyloidosis. In addition, two clinical cases and practical tips are provided in this context.Keywords: cardiac amyloidosis, ATTR amyloidosis, AL amyloidosis, echocardiography, cardiac magnetic resonance, bone scintigraphy, myocardial biopsy, specific therapy

Published
2021

19. Two-Year Evaluation of the German Clinical Amyloidosis Registry

Author

Hegenbart, Ute, primary, Fuhr, Niklas, additional, Huber, Laura, additional, Kimmich, Christoph, additional, Veelken, Kaya, additional, Dittrich, Tobias, additional, Ziehl, Rita, additional, aus dem Siepen, Fabian, additional, Carpinteiro, Alexander, additional, Hansen, Timon, additional, Müller-Tidow, Carsten, additional, Benner, Axel, additional, and Schönland, Stefan, additional

Published
2021
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30. Long-term safety and efficacy of patisiran for hereditary transthyretin-mediated amyloidosis with polyneuropathy: 12-month results of an open-label extension study

Author

Adams, David, primary, Polydefkis, Michael, additional, González-Duarte, Alejandra, additional, Wixner, Jonas, additional, Kristen, Arnt V, additional, Schmidt, Hartmut H, additional, Berk, John L, additional, Losada López, Inés Asunción, additional, Dispenzieri, Angela, additional, Quan, Dianna, additional, Conceição, Isabel M, additional, Slama, Michel S, additional, Gillmore, Julian D, additional, Kyriakides, Theodoros, additional, Ajroud-Driss, Senda, additional, Waddington-Cruz, Márcia, additional, Mezei, Michelle M, additional, Planté-Bordeneuve, Violaine, additional, Attarian, Shahram, additional, Mauricio, Elizabeth, additional, Brannagan, Thomas H, additional, Ueda, Mitsuharu, additional, Aldinc, Emre, additional, Wang, Jing Jing, additional, White, Matthew T, additional, Vest, John, additional, Berber, Erhan, additional, Sweetser, Marianne T, additional, Coelho, Teresa, additional, Vita, Giuseppe, additional, Rizzo, Vincenzo, additional, Russo, Massimo, additional, Mazzeo, Anna, additional, Gentile, Luca, additional, Brueckner, Caitlin, additional, Lazzari, Victoria, additional, Wiesman, Janice, additional, DeLong, Douglas, additional, Victory, Jennifer, additional, Dalton, James, additional, May, John, additional, Gilmore, Catherine, additional, Diallo, Saran, additional, Delmont, Emilien, additional, Pouget, Jean, additional, Verschueren, Annie, additional, Grapperon, Aude-Marie, additional, Campana-Salort, Emmanuelle, additional, Lopes, Ana, additional, Lamas, Filipa, additional, Neves, Carlos, additional, Castro, Jose, additional, Pereira, Pedro, additional, Castro, Isabel, additional, Franco, Ana, additional, Santos, Miguel Oliveira, additional, de Azevedo Coutinho, Conceição, additional, Falcao de Campos, Catarina, additional, Hipólito Reis, Antonio, additional, Correia, Nuno, additional, Perez, Javier M, additional, Martins da Silva, Ana, additional, Alves, Cristina, additional, Cardoso, Marcio, additional, Valdrez, Katia, additional, Monte, Julia R, additional, Pessoa, Bernardete, additional, Guimaraes, Nadia, additional, Freitas, Monica, additional, Ramalho, Joana, additional, Ferreira, Natalia, additional, Kuzume, Daisuke, additional, Tard, Celine, additional, Waucquier, Nawal, additional, Rougeaux, Isabelle, additional, Brice, Sylvie, additional, Kasprzyk, Emmanuelle, additional, Elrezzi, Elise, additional, Meguig, Sayah, additional, Hachulla, Eric, additional, Gauvain, Clement, additional, Migaud-Chervy, Maria-Claire, additional, Deplanque, Dominique, additional, Jozefowicz, Elsa, additional, Lebellec, Loic, additional, Adams, David, additional, Balaya-Gouraya, Line, additional, Jehan Lacour, Nathalie, additional, Bournane, Halima, additional, Martin, Nathalie, additional, Elabed, Mongia, additional, Sacko, Niamey, additional, Boubrit, Yasmine, additional, Gaouar, Amina, additional, Rakotondratafika, Fetra, additional, Théaudin-Saliou, Marie, additional, Cauquil-Michon, Cécile, additional, Labeyrie, Celine, additional, Not, Adeline, additional, Al-Salameh, Abdallah, additional, Lecoq, Anne-Lise, additional, Stephant, Maeva, additional, Echaniz-Laguna, Andoni, additional, Becquemont, Laurent, additional, Beaudonnet, Guillemette, additional, Algalarrondo, Vincent, additional, Eliahou, Ludivine, additional, Rousseau, Antoine, additional, Signate, Aissatou, additional, Berthelot, Emeline, additional, Inamo, Jocelyn, additional, Vervoitte, Laetitia, additional, Focseneanu, Cecile, additional, Gendre, Thierry, additional, Arrouasse, Raphaele, additional, Ayache, Samar S., additional, Ernande, Laura, additional, Le Corvoisier, Philippe, additional, Salhi, Hayet, additional, Choumert, Ariane, additional, Ehinger, Vincent, additional, Ruiz, Julie, additional, Charlin, Cyril, additional, Megelin, Thomas, additional, Brannagan III, Thomas H, additional, Fayerman, Raisy, additional, Kim, Arreum, additional, Paras, Allan, additional, Gonzalez, Leidy J, additional, Tsang, Steven, additional, Wajnsztajn, Fernanda, additional, Shije, Jeffrey, additional, Ulane, Christina, additional, Kleyman, Inna, additional, Weimer, Louis, additional, Cioroiu, Comana, additional, Lambrianides, Sakis, additional, Abu-Manneh, Rana, additional, Zamba-Papanicolaou, Eleni, additional, Agathangelou, Petros, additional, Leonidou, Eleni, additional, Tada, Satoshi, additional, Fujita, Akemi, additional, Nagai, Masahiro, additional, Ando, Rina, additional, Hosokawa, Yuko, additional, Yamanishi, Yuki, additional, Overcash, J. Scott, additional, Giardino, Elena, additional, Boyer, Leslie, additional, Dang, Lien, additional, Le, An, additional, Nguyen, Tyler, additional, Giang, Lien, additional, Sellers, Peter, additional, Tran, Leyla, additional, Truong, Nghi, additional, Vinas, Maita, additional, Hrkman, Nicole, additional, Miller, Sarah, additional, Nguyen, David, additional, Smith, Ashley, additional, Pu, Helen, additional, Li, Steve, additional, Vuong, Thao, additional, Dioso, Holly, additional, Green, Sinikka, additional, Lee, Kia, additional, Chu, Hanh, additional, Waters, Michael, additional, Coskun, Derya J, additional, Zepeda, Karla A, additional, O'Riordan, William, additional, Obici, Laura, additional, Cortese, Andrea, additional, Lozza, Alessandro, additional, Merlini, Giampaolo, additional, Rosti, Vittorio, additional, Sabatelli, Mario, additional, Bisogni, Giulia, additional, Bernardo, Daniela, additional, Luigetti, Marco, additional, Di Paolantonio, Andrea, additional, Guglielmino, Valeria, additional, Romano, Angela, additional, Nienhuis, Hans, additional, Bulthuis-Kuiper, Janita, additional, Gerk, Olga, additional, Ulbricht, Hannah, additional, Taylor, Lenka, additional, Meyle, Eva, additional, Kleinschmidt, Natalia, additional, Meyrath, David, additional, Noe-Schwenn, Simone, additional, Meng, Ulrike, additional, Bauer, Ralf, additional, aus dem Siepen, Fabian, additional, Hein, Selina, additional, Takahashi, Tetsuya, additional, Oshita, Tomohiko, additional, Koujin, Yoko, additional, Neshige, Shuichiro, additional, Nezu, Tomohisa, additional, Segawa, Akiko, additional, Ueno, Hiroki, additional, Morino, Hiroyuki, additional, Campistol, Josep M, additional, Rodas Marin, Lida Maria, additional, Blasco Pelicano, Josep Miquel, additional, Dávila, Lucía Galán, additional, Palacios, Marta, additional, Pytel Cordoba, Vanesa, additional, Guerrero Sola, Antonio, additional, Horga, Alejandro, additional, García Feijoo, Julián, additional, Perez de Isla, Leopoldo, additional, Marques Júnior, Wilson, additional, Moscardini, Mariana, additional, Litcanov, Debora Cristina, additional, Viera Lima, Ana Flavia, additional, Rodrigues, Leonardo, additional, Marques Coutinho, Barbara, additional, Moreira, Carolina Lavigne, additional, Daccach Marques, Vanessa, additional, Munoz Beamud, Francisco, additional, Gragera Martínez, Álvaro, additional, Borrachero, Cristina, additional, Cisneros Barroso, Eugenia, additional, Rodríguez Rodríguez, Adrián, additional, Sanz, Monica, additional, Rigo Oliver, Elena, additional, González Moreno, Juan, additional, Gamez Martinez, Jose M, additional, Descals, Cristina, additional, Uson, Mercedes, additional, Jose Vega, Francisco, additional, Figuerola, Antoni, additional, Montala, Carles, additional, Dias da Silva, Moises, additional, Gervais de Santa Rosa, Renata, additional, Pinto, Luiz Felipe, additional, Pinto, Marcus Vinicius, additional, Cardoso Berensztejn, Amanda, additional, Barroso, Fabio, additional, Lautre, Andrea, additional, Orellana, Lucas G, additional, González-Duarte Briseño, Maria Alejandra, additional, Cárdenas-Soto, Karla, additional, Jiménez López, Brenda Poled, additional, Pérez-Castañeda, Sandra Lorena, additional, Cantú Brito, Carlos Gerardo, additional, Rivera de la Parra, David, additional, Hernandez Reyes, Jose Pablo, additional, del Mar Saniger Alba, Maria, additional, Criollo Mora, Elia, additional, Parman, Yesim, additional, Rezzan, Kus Jülide, additional, Sahin, Erdi, additional, Serbest, Nail G, additional, Durmus, Hacer, additional, Cakar, Arman, additional, Tugal Tutkun, Nuriye Ilknur, additional, Karamursel, Sacit, additional, Elitok, Ali, additional, Sirin Inan, Nermin G, additional, Altinkurt, Emre, additional, Ye, Jing, additional, Allen, Adriane C, additional, Chaudhry, Vinay, additional, Jarrett, Raquel, additional, Bressler, Neil, additional, Burks, Kathleen L, additional, Liu, Qingfeng, additional, Khoshnoodi, Mohammad, additional, Judge, Daniel P, additional, Vista, Geno, additional, Shah, Syed Mahmood, additional, Hamaguchi, Hirotoshi, additional, Oda, Junko, additional, Fukase, Emi, additional, Taniguchi, Ikuko, additional, Oda, Tetsuya, additional, Endo, Hironobu, additional, Shimomura, Masahiro, additional, Katanazaka, Kimitaka, additional, Koto, Shusuke, additional, Nakano, Takahiro, additional, Scheid, Christof, additional, Zueiter, Andreas, additional, Pester, Lars, additional, Walter, Doreen, additional, Özdemir, Betül, additional, Frenzel, Lukas F, additional, Holtick, Udo, additional, Oh, Jeeyoung, additional, Kim, Hee Jin, additional, Shin, Hyun Jin, additional, Choi, Kyomin, additional, Yamashita, Taro, additional, Masuda, Teruaki, additional, Misumi, Yohei, additional, Ueda, Akihiko, additional, Nakahara, Keiichi, additional, Yorita, Akiko, additional, Tsuruhisa, Seiko, additional, Taniwaki, Takayuki, additional, Harada, Masaya, additional, Moritaka, Taiga, additional, Sakurada, Naonori, additional, Mauricio, Elizabeth A, additional, Baskin, Amber, additional, Dimberg, Elliot, additional, Fonder, Amie, additional, Hobbs, Miriam, additional, Russell, Stephen J, additional, Dyck, Peter, additional, Gonsalves, Wilson, additional, Leung, Nelson, additional, Witzig, Thomas E, additional, Zeldenrust, Steven R, additional, Hwa, Lisa, additional, Kapoor, Prashant, additional, Kumar, Shaji K, additional, Lin, Yi, additional, Lust, John A, additional, Rajkumar, Vincent S, additional, Dingli, David, additional, Gertz, Morie A, additional, Go, Ronald, additional, Hayman, Suzanne R, additional, Dalia, Samir, additional, Carrillo, Esmeralda, additional, Gorevic, Peter, additional, Mason, Garnette, additional, Chao, Chi-Chao, additional, Lee, Ming-Jen, additional, Su, Jen-Jen, additional, Hsieh, Sung-Tsang, additional, Tsai, Li-Kai, additional, Yeh, Shin-Joe, additional, Yang, Chih-Chao, additional, Ajroud-Driss, Senda Ajroud-Driss, additional, Casey, Patricia, additional, Joslin, Benjamin C, additional, Freimer, Miriam, additional, Sankey, Alison, additional, Kenepp, Amanda, additional, Heintzman, Sarah, additional, LoRusso, Samantha, additional, Hokezu, Youichi, additional, Kim, Byoung-Joon, additional, Kim, JuHyeon, additional, Lee, Ga Yeon, additional, Cho, Eun Bin, additional, Jeon, Eun-Seok, additional, Min, Ju-Hong, additional, Seok, Jin Myoung, additional, Lee, Hye Lim, additional, Park, Jae Hong, additional, Sekijima, Yoshiki, additional, Miyazawa, Chinatsu, additional, Kato, Nagaaki, additional, Kishida, Dai, additional, Hineno, Akiyo, additional, Kodaira, Minori, additional, Yoshinaga, Tsuneaki, additional, Miyahara, Teruyoshi, additional, Imai, Akira, additional, Matsumoto, Kazuhiko, additional, Lin, Kon-Ping, additional, Lee, Yi-Chung, additional, Falk, Malin, additional, Pilebro, Bjorn, additional, Suhr, Ole, additional, Lindqvist, Per, additional, Soderberg, Karin, additional, Pedrosa-Domellöf, Fatima, additional, Anan, Intissar, additional, Nordh, Erik, additional, Tournev, Ivaylo, additional, Zhelyazkova-Glaveeva, Sashka, additional, Cherneva, Zheyna, additional, Sarafov, Staiko, additional, Chamova, Teodora, additional, Cherninkova-Gopina, Sylvia, additional, Friebel, Frauke, additional, Zibert, Andree, additional, Mihailovic, Natasa, additional, Schubert, Friederike, additional, Vorona, Elena, additional, Lahme, Larissa, additional, Huesing-Kabar, Anna, additional, Schilling, Matthias, additional, Kabar, Iyad, additional, Martinez-Naharro, Ana, additional, Chacko, Liza, additional, Cohen, Oliver, additional, Law, Steven, additional, Rezk, Tamer, additional, Lachmann, Helen J, additional, Blume, Brianna, additional, Dixon, Stacy, additional, Low, Soon Chai, additional, Chan, Soo Looi, additional, Lim, He Eng Li, additional, Goh, Khean Jin, additional, Kraus, Deborah, additional, Jack, Kristin, additional, Wade, N. Kevin, additional, Lopate, Glenn, additional, Zwijack, Brittany, additional, Florence, Julaine, additional, Sommerville, R. Brian, additional, Stewart, Graeme, additional, Ryder, Julie, additional, Mekhael, Linda, additional, Taylor, Mark, additional, Suan, Daniel, additional, Wells, Karen, additional, Stone, Paula, additional, Itoya, Amenze, additional, Owusu-Sekyere, Mercy, additional, Thai, Desmond, additional, Chahine, Ilonah, additional, Pedrosa, Salve, additional, and Do, Thi Hoa (Therese), additional

Published
2021
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31. Real-world outcomes in non-endemic hereditary transthyretin amyloidosis with polyneuropathy: a 20-year German single-referral centre experience

Author

Ungerer, Matthias N., primary, Hund, Ernst, additional, Purrucker, Jan C., additional, Huber, Laura, additional, Kimmich, Christoph, additional, aus dem Siepen, Fabian, additional, Hein, Selina, additional, Kristen, Arnt V., additional, Hinderhofer, Katrin, additional, Kollmer, Jennifer, additional, Schönland, Stefan, additional, Hegenbart, Ute, additional, and Weiler, Markus, additional

Published
2020
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32. Real-world outcomes in non-endemic hereditary transthyretin amyloidosis with polyneuropathy: a 20-year German single-referral centre experience.

Author

Ungerer, Matthias N., Hund, Ernst, Purrucker, Jan C., Huber, Laura, Kimmich, Christoph, aus dem Siepen, Fabian, Hein, Selina, Kristen, Arnt V., Hinderhofer, Katrin, Kollmer, Jennifer, Schönland, Stefan, Hegenbart, Ute, and Weiler, Markus

Subjects
CARDIAC amyloidosis, AMYLOIDOSIS, TRANSTHYRETIN, POLYNEUROPATHIES, NEUROLOGICAL disorders, BRAIN natriuretic factor
Abstract

Hereditary transthyretin amyloidosis is caused by pathogenic variants in the TTR gene and typically manifests, alongside cardiac and other organ dysfunctions, with a rapidly progressive sensorimotor and autonomic polyneuropathy (ATTRv-PN) leading to severe disability. While most prospective studies have focussed on endemic ATTRv-PN, real-world data on non-endemic, mostly late-onset ATTRv-PN are limited. This retrospective study investigated ATTRv-PN patients treated at the Amyloidosis Centre of Heidelberg University Hospital between November 1999 and July 2020. Clinical symptoms, survival, prognostic factors and efficacy of treatment with tafamidis were analysed. Neurologic outcome was assessed using the Coutinho ATTRv-PN stages, and the Peripheral Neuropathy Disability (PND) score. Of 346 subjects with genetic TTR variants, 168 patients had symptomatic ATTRv-PN with 32 different TTR variants identified. Of these, 81.6% had the late-onset type of ATTRv-PN. Within a mean follow-up period of 4.1 ± 2.8 years, 40.5% of patients died. Baseline plasma N-terminal prohormone of brain natriuretic peptide (NT-proBNP) ≥900 ng/l (HR 3.259 [1.421–7.476]; p =.005) was the main predictor of mortality in multivariable analysis. 64 patients were treated with tafamidis and presented for regular follow-up examinations. The therapeutic benefit of tafamidis was more pronounced when treatment was started early in ATTRv-PN stage 1 (PND scores II vs. I; HR 2.718 [1.258–5.873]; p =.011). In non-endemic, mostly late-onset ATTRv-PN, cardiac involvement assessed by NT-proBNP is a strong prognosticator for overall survival. Long-term treatment with tafamidis is safe and efficacious. Neurologic disease severity at the start of treatment is the main predictor for ATTRv-PN progression on tafamidis. [ABSTRACT FROM AUTHOR]

Published
2021
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34. Variability of cardiovascular magnetic resonance (CMR) T1 mapping parameters in healthy volunteers during long-term follow-up

Author

aus dem Siepen, Fabian, primary, Baumgärtner, Christian, additional, Müller-Henessen, Matthias, additional, André, Florian, additional, Messroghli, Daniel, additional, Ochs, Marco, additional, Riffel, Johannes, additional, Giannitsis, Evangelos, additional, Katus, Hugo A, additional, Friedrich, Matthias G, additional, and Buss, Sebastian J, additional

Published
2018
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35. Diagnostic and Prognostic Value of Long-Axis Strain and Myocardial Contraction Fraction Using Standard Cardiovascular MR Imaging in Patients with Nonischemic Dilated Cardiomyopathies

Author

Arenja, Nisha, primary, Riffel, Johannes H., additional, Fritz, Thomas, additional, André, Florian, additional, aus dem Siepen, Fabian, additional, Mueller-Hennessen, Matthias, additional, Giannitsis, Evangelos, additional, Katus, Hugo A., additional, Friedrich, Matthias G., additional, and Buss, Sebastian J., additional

Published
2017
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37. Reply

Author

Kristen, Arnt V., primary, Brokbals, Eva, additional, aus dem Siepen, Fabian, additional, Bauer, Ralf, additional, Hein, Selina, additional, Aurich, Matthias, additional, Riffel, Johannes, additional, Behrens, Hans-Michael, additional, Krüger, Sandra, additional, Schirmacher, Peter, additional, Katus, Hugo A., additional, and Röcken, Christoph, additional

Published
2016
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38. Cardiac Amyloid Load

Author

Kristen, Arnt V., primary, Brokbals, Eva, additional, aus dem Siepen, Fabian, additional, Bauer, Ralf, additional, Hein, Selina, additional, Aurich, Matthias, additional, Riffel, Johannes, additional, Behrens, Hans-Michael, additional, Krüger, Sandra, additional, Schirmacher, Peter, additional, Katus, Hugo A., additional, and Röcken, Christoph, additional

Published
2016
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39. Left ventricular long axis strain: a new prognosticator in non-ischemic dilated cardiomyopathy?

Author

Riffel, Johannes H., primary, Keller, Marius G. P., additional, Rost, Franziska, additional, Arenja, Nisha, additional, Andre, Florian, additional, aus dem Siepen, Fabian, additional, Fritz, Thomas, additional, Ehlermann, Philipp, additional, Taeger, Tobias, additional, Frankenstein, Lutz, additional, Meder, Benjamin, additional, Katus, Hugo A., additional, and Buss, Sebastian J., additional

Published
2016
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40. Left ventricular mechanics assessed by two-dimensional echocardiography and cardiac magnetic resonance imaging: comparison of high-resolution speckle tracking and feature tracking

Author

Aurich, Matthias, primary, Keller, Marius, additional, Greiner, Sebastian, additional, Steen, Henning, additional, aus dem Siepen, Fabian, additional, Riffel, Johannes, additional, Katus, Hugo A., additional, Buss, Sebastian J., additional, and Mereles, Derliz, additional

Published
2016
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41. Green tea extract as a treatment for patients with wild-type transthyretin amyloidosis: an observational study

Author

aus dem Siepen,Fabian, Bauer,Ralf, Aurich,Matthias, Buss,Sebastian J, Steen,Henning, Altland,Klaus, Katus,Hugo A, Kristen,Arnt V, aus dem Siepen,Fabian, Bauer,Ralf, Aurich,Matthias, Buss,Sebastian J, Steen,Henning, Altland,Klaus, Katus,Hugo A, and Kristen,Arnt V

Abstract

Fabian aus dem Siepen,1 Ralf Bauer,1 Matthias Aurich,1 Sebastian J Buss,1 Henning Steen,1 Klaus Altland,2 Hugo A Katus,1 Arnt V Kristen1 1Department of Cardiology, Angiology, and Respiratory Medicine, University Hospital Heidelberg, Heidelberg, Germany; 2Institute of Human Genetics, Justus-Liebig-University, Giessen, Germany Background: Causative treatment of patients with wild-type transthyretin amyloid cardiomyopathy (wtATTR-CM) is lacking. Recent reports indicate the potential use of epigallocatechin-3-gallate (EGCG), the most abundant catechin in green tea, to inhibit amyloid fibril formation. We sought to investigate changes of cardiac function and morphology in patients with wtATTR-CM after consumption of green tea extract (GTE). Methods: Twenty-five male patients (71 [64; 80] years) with wtATTR-CM were submitted to clinical examination, echocardiography, cardiac magnetic resonance imaging (cMRI) (n=14), and laboratory testing before and after daily consumption of GTE capsules containing 600 mg epigallocatechin-3-gallate for at least 12 months. Results: A significant decrease of left ventricular (LV) myocardial mass by 6% (196 [100; 247] vs 180 [85; 237] g; P=0.03) by cMRI and total cholesterol by 8.4% (191 [118; 267] vs 173 [106; 287] mg/dL; P=0.006) was observed after a 1-year period of GTE consumption. LV ejection fraction by cMRI (53% [33%; 69%] vs 54% [28%; 71%]; P=0.75), LV wall thickness (17 [13; 21] vs 18 [14; 25] mm; P=0.1), and mitral annular plane systolic excursion (10 [5; 23] vs 8 [4; 13] mm; P=0.3) by echocardiography remained unchanged. Conclusion: This study supports LV mass stabilization in patients with wtATTR-CM consuming GTE potentially indicating amyloid fibril reduction. Keywords: wild-type ATTR, cardiomyopathy, polyphenol, EGCG

Published
2015

43. Peak V'O2 is an independent predictor of survival in patients with cardiac amyloidosis.

Author

Hein, Selina, Aus Dem Siepen, Fabian, Bauer, Ralf, Katus, Hugo A., and Kristen, Arnt V.

Subjects
*CARDIAC amyloidosis, *CARDIOPULMONARY fitness, *HEART failure, *MORTALITY, *ECHOCARDIOGRAPHY
Abstract

Introduction: Cardiopulmonary exercise testing (CPET) has repeatedly been reported to reliably predict adverse outcomes in different forms of heart failure. However, it has not been elucidated in detail in cardiac amyloidosis (CA). Therefore, we evaluated the predictive value of CPET parameters in patients with CA regarding disease severity and prediction of mortality. Methods: Twenty-seven consecutive patients with CA were assessed noninvasively, including electrocardiography, echocardiography, CPET, and laboratory tests. Clinical data were correlated with CPET findings. Univariate and multivariate analyses were performed to evaluate predictors of mortality. Results: Within median follow-up period of 38 (IQR 43) months 19 (70%) deaths occurred. Patient initially presented with signs and symptoms of congestive heart failure NYHA 3 (IQR 1), reduced exercise capacity (peak V'O2 15.2 mL/kg body weight) and inefficient ventilation in CPET (V'E/V'CO2 slope (30 (IQR 3)), markedly elevated cardiac biomarkers (NT-proBNP 1791 (IQR 3249) ng/mL) and echocardiographic signs of morphological (septum thickness 18 (IQR 6) mm) and functional cardiac involvement (TAPSE 19 (IQR 8) mm). Patients with peak V'O2 below median value presented with significantly longer QTc interval when compared to patients with peak V'O2 above the median. Further these patients tend to have more pronounced impairment of longitudinal function as indicated by lower MAPSE, TAPSE, and elevation of cardiac biomarkers. Multivariate analysis revealed peak V'O2 slope as the only independent predictor of survival. Conclusions: We identified reduced peak V'O2 as an independent predictor of mortality in patients with cardiac involvement in different forms of systemic amyloidosis. [ABSTRACT FROM AUTHOR]

Published
2018
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44. Comparison of different types of cardiac amyloidosis by cardiac magnetic resonance imaging

Author

Kristen, Arnt V., primary, aus dem Siepen, Fabian, additional, Scherer, Katrin, additional, Kammerer, Rebekka, additional, Andre, Florian, additional, Buss, Sebastian J., additional, Bauer, Ralf, additional, Lehrke, Stephanie, additional, Voss, Andreas, additional, Giannitsis, Evangelos, additional, Katus, Hugo A., additional, and Steen, Henning, additional

Published
2015
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46. Noninvasive Risk Stratification of Patients With Transthyretin Amyloidosis

Author

Kristen, Arnt V., primary, Scherer, Katrin, additional, Buss, Sebastian, additional, aus dem Siepen, Fabian, additional, Haufe, Sabine, additional, Bauer, Ralf, additional, Hinderhofer, Katrin, additional, Giannitsis, Evangelos, additional, Hardt, Stefan, additional, Haberkorn, Uwe, additional, Katus, Hugo A., additional, and Steen, Henning, additional

Published
2014
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48. T2-relaxometry in a large cohort of hereditary transthyretin amyloidosis with polyneuropathy.

Author

Poncelet, Anysia, Hegenbart, Ute, Schönland, Stefan O., Sam, Georges, Purrucker, Jan C., Hund, Ernst, aus dem Siepen, Fabian, Göldner, Kira, Hayes, John M., Heiland, Sabine, Bendszus, Martin, Weiler, Markus, and Hayes, Jennifer C.

Subjects
*MAGNETIC resonance neurography, *NERVE conduction studies, *PERONEAL nerve, *PERIPHERAL nervous system, *TIBIAL nerve
Abstract

AbstractBackgroundMethodsResultsConclusionPreviously, T2-relaxation time (T2app) and proton spin density (ρ) detected nerve injury in a small group of ATTRv amyloidosis. Here, we aim to quantify peripheral nerve impairment in a large cohort of symptomatic and asymptomatic ATTRv amyloidosis and correlate T2-relaxometry markers with clinical parameters and nerve conduction studies (NCS).Eighty participants with pathologic variants of the transthyretin gene (TTRv) and 40 controls prospectively underwent magnetic resonance neurography. T2-relaxometry was performed, allowing to calculate tibial ρ, T2app and cross-sectional-area (CSA). Detailed clinical examinations and NCS of tibial and peroneal nerves were performed.Forty participants were classified as asymptomatic TTRv-carriers, 40 as symptomatic patients with polyneuropathy. ρ, T2app and CSA were significantly higher in symptomatic ATTRv amyloidosis (484.2 ± 14.8 a.u.; 70.6 ± 1.8 ms; 25.7 ± 0.9 mm2) versus TTRv-carriers (413.1 ± 9.4 a.u., p < 0.0001; 62.3 ± 1.3 ms, p = 0.0002; 19.0 ± 0.8 mm2, p < 0.0001) and versus controls (362.6 ± 7.5 a.u., p < 0.0001; 59.5 ± 1.0 ms, p < 0.0001; 15.4 ± 0.5 mm2, p < 0.0001). Only ρ and CSA differentiated TTRv-carriers from controls. ρ and CSA correlated with NCS in TTRv-carriers, while T2app correlated with NCS in symptomatic ATTRv amyloidosis. Both ρ and T2app correlated with clinical score.ρ and CSA can detect early nerve injury and correlate with electrophysiology in asymptomatic TTRv-carriers. T2app increases only in symptomatic ATTRv amyloidosis in whom it correlates with clinical scores and electrophysiology. Our results suggest that T2-relaxometry can provide biomarkers for disease- and therapy-monitoring in the future. [ABSTRACT FROM AUTHOR]

Published
2024
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49. Cardiac Amyloid Load: A Prognostic and Predictive Biomarker in Patients With Light-Chain Amyloidosis.

Author

Kristen, Arnt V., Brokbals, Eva, aus dem Siepen, Fabian, Bauer, Ralf, Hein, Selina, Aurich, Matthias, Riffel, Johannes, Behrens, Hans-Michael, Krüger, Sandra, Schirmacher, Peter, Katus, Hugo A., and Röcken, Christoph

Subjects
*AMYLOIDOSIS, *BIOMARKERS, *MORTALITY, *HISTOLOGY, *ELECTROCARDIOGRAPHY, *RETROSPECTIVE studies, *PREVENTION
Abstract

Background: Cardiac amyloid load has not been analyzed for its effect on mortality in patients with amyloid light-chain (AL) cardiac amyloidosis.Objectives: This study retrospectively compared histological amyloid load with common clinical predictors of mortality.Methods: This study assessed 216 patients with histologically confirmed cardiac amyloidosis at a single center with electrocardiography, echocardiography, and laboratory testing.Results: AL amyloid deposits were usually distributed in a reticular/pericellular pattern, whereas transthyretin amyloid (ATTR) more commonly showed patchy deposits. Median amyloid load was 30.5%; no amyloid load was above 70%. During follow-up (median 19.1 months), 112 patients died. Chemotherapy had a significant effect on overall survival in AL amyloidosis (16.2 months vs. 1.4 months; p = 0.003). Patients with <20% AL amyloid load who responded to chemotherapy showed significantly better survival than nonresponders. According to univariate analysis, predictors of survival in AL amyloidosis included sex, Karnofsky index, New York Heart Association (NYHA) functional class, diastolic blood pressure, estimated glomerular filtration rate, N-terminal pro-B-type natriuretic peptide, mineralocorticoid receptor antagonists, low voltage, ineligibility for chemotherapy, response to chemotherapy, and amyloid load. Independent predictors of mortality by multivariate analysis included NYHA functional class (III vs. II), estimated glomerular filtration rate, responders to chemotherapy, and amyloid load. In ATTR amyloidosis, survival correlated with NYHA functional class, diastolic blood pressure, and use of diuretic agents. Following Cox regression analysis, NYHA functional class (III vs. II; p < 0.05) remained the only independent predictor of patient survival in ATTR amyloidosis.Conclusions: Early identification of subjects with AL amyloid is essential given that in late-stage disease with extensive amyloid load, our data suggested that outcomes are not affected by administration of chemotherapy. [ABSTRACT FROM AUTHOR]

Published
2016
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50. Reply: Should Histologic Determination of Amyloid Load Determine Management Decisions in Light-Chain Amyloidosis?

Author

Kristen, Arnt V., Brokbals, Eva, aus dem Siepen, Fabian, Bauer, Ralf, Hein, Selina, Aurich, Matthias, Riffel, Johannes, Behrens, Hans-Michael, Krüger, Sandra, Schirmacher, Peter, Katus, Hugo A., and Röcken, Christoph

Subjects
*AMYLOIDOSIS diagnosis, *AMYLOIDOSIS treatment, *HISTOLOGY, *MEDICAL decision making, *CARDIAC research, *AMYLOIDOSIS, *DECISION making, *PROTEINS
Published
2016
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