Search

Your search keyword '"Kristen, Arnt V"' showing total 503 results
Start Over Author "Kristen, Arnt V"
503 results on '"Kristen, Arnt V"'

53. Sex-Related Risk of Cardiac Involvement in Hereditary Transthyretin Amyloidosis

Author

Caponetti, Angelo Giuseppe, primary, Rapezzi, Claudio, additional, Gagliardi, Christian, additional, Milandri, Agnese, additional, Dispenzieri, Angela, additional, Kristen, Arnt V., additional, Wixner, Jonas, additional, Maurer, Mathew S., additional, Garcia-Pavia, Pablo, additional, Tournev, Ivailo, additional, Planté-Bordeneuve, Violaine, additional, Chapman, Douglass, additional, Amass, Leslie, additional, van Cleemput, Johan, additional, Waddington-Cruz, Marcia, additional, Schmidt, Hartmut, additional, Dillmann, Klaus-Ulrich, additional, Mølgaard, Henning, additional, Moreno, Juan Gonzalez, additional, Costello, Jose Gonzalez, additional, Beamud, Francisco Munoz, additional, Davila, Lucia Galan, additional, Adams, David, additional, Inamo, Jocelyn, additional, Lairez, Olivier, additional, Vita, Giuseppe, additional, Merlini, Giampaolo, additional, Cirami, Calogero Lino, additional, Luigetti, Marco, additional, Emdin, Michele, additional, Sekijima, Yoshiki, additional, Jeon, Eun-Seok, additional, Oh, Jeeyoung, additional, Gonzalez Duarte Briseno, Maria Alejandra, additional, Nienhuis, Hans, additional, Coelho, Teresa, additional, Conceicao, Isabel, additional, Azevedo, Olga, additional, Badelita, Sorina, additional, Press, Rayomand, additional, Parman, Yesim, additional, Shah, Sanjiv, additional, Quan, Dianna, additional, Marburger, Tessa, additional, Polydefkis, Michael, additional, Witteles, Ronald, additional, Gottlieb, Stephen, additional, Sarswat, Nitasha, additional, Drachman, Brian, additional, Steidley, David, additional, Hummel, Scott, additional, Slosky, David, additional, Jacoby, Daniel, additional, Nativi-Nicolau, Jose, additional, Tauras, James, additional, Zivkovic, Sasa, additional, Tallaj, Jose, additional, and Lenihan, Daniel, additional

Published
2021
Full Text
View/download PDF

54. Temporal Trends of Wild-Type Transthyretin Amyloid Cardiomyopathy in the Transthyretin Amyloidosis Outcomes Survey

Author

Nativi-Nicolau, Jose, primary, Siu, Alfonso, additional, Dispenzieri, Angela, additional, Maurer, Mathew S., additional, Rapezzi, Claudio, additional, Kristen, Arnt V., additional, Garcia-Pavia, Pablo, additional, LoRusso, Samantha, additional, Waddington-Cruz, Márcia, additional, Lairez, Olivier, additional, Witteles, Ronald, additional, Chapman, Doug, additional, Amass, Leslie, additional, Grogan, Martha, additional, Barroso, Fabio Adrian, additional, Van Cleemput, Johan, additional, Fine, Nowell, additional, Schmidt, Hartmut, additional, Gess, Burkhard, additional, Moelgaard, Henning, additional, Planté-Bordeneuve, Violaine, additional, Adams, David, additional, Inamo, Jocelyn, additional, Vita, Giuseppe, additional, Cirami, Calogero Lino, additional, Luigetti, Marco, additional, Emdin, Michele, additional, Sekijima, Yoshiki, additional, Yamashita, Taro, additional, Jeon, Eun-Seok, additional, Gonzalez Duarte Briseno, Maria Alejandra, additional, Nienhuis, Hans, additional, Azevedo, Olga, additional, Campistol Plana, Josep Maria, additional, Moreno, Juan Gonzalez, additional, Costello, Jose Gonzalez, additional, Wixner, Jonas, additional, Parman, Yesim, additional, Shah, Sanjiv, additional, Quan, Dianna, additional, Marburger, Tessa, additional, Polydefkis, Michael, additional, Gottlieb, Stephen, additional, Ralph, Jeffrey, additional, Sarswat, Nitasha, additional, Luo, Jin, additional, Murali, Srinivas, additional, Cotts, William, additional, Drachman, Brian, additional, Steidley, David, additional, Hummel, Scott, additional, Slosky, David, additional, Ventura, Hector, additional, Jacoby, Daniel, additional, Hoffman, James, additional, Tauras, James, additional, Zivkovic, Sasa, additional, Tallaj, Jose, additional, Lenihan, Daniel, additional, and Mueller, Christopher, additional

Published
2021
Full Text
View/download PDF

57. ASNC/AHA/ASE/EANM/HFSA/ISA/SCMR/SNMMI Expert Consensus Recommendations for Multimodality Imaging in Cardiac Amyloidosis: Part 1 of 2—Evidence Base and Standardized Methods of Imaging

Author

Dorbala, Sharmila, primary, Ando, Yukio, additional, Bokhari, Sabahat, additional, Dispenzieri, Angela, additional, Falk, Rodney H., additional, Ferrari, Victor A., additional, Fontana, Marianna, additional, Gheysens, Olivier, additional, Gillmore, Julian D., additional, Glaudemans, Andor W. J. M., additional, Hanna, Mazen A., additional, Hazenberg, Bouke P. C., additional, Kristen, Arnt V., additional, Kwong, Raymond Y., additional, Maurer, Mathew S., additional, Merlini, Giampaolo, additional, Miller, Edward J., additional, Moon, James C., additional, Murthy, Venkatesh L., additional, Quarta, C. Cristina, additional, Rapezzi, Claudio, additional, Ruberg, Frederick L., additional, Shah, Sanjiv J., additional, Slart, Riemer H. J. A., additional, Verberne, Hein J., additional, and Bourque, Jamieson M., additional

Published
2021
Full Text
View/download PDF

58. ASNC/AHA/ASE/EANM/HFSA/ISA/SCMR/SNMMI Expert Consensus Recommendations for Multimodality Imaging in Cardiac Amyloidosis: Part 2 of 2—Diagnostic Criteria and Appropriate Utilization

Author

Dorbala, Sharmila, primary, Ando, Yukio, additional, Bokhari, Sabahat, additional, Dispenzieri, Angela, additional, Falk, Rodney H., additional, Ferrari, Victor A., additional, Fontana, Marianna, additional, Gheysens, Olivier, additional, Gillmore, Julian D., additional, Glaudemans, Andor W. J. M., additional, Hanna, Mazen A., additional, Hazenberg, Bouke P. C., additional, Kristen, Arnt V., additional, Kwong, Raymond Y., additional, Maurer, Mathew S., additional, Merlini, Giampaolo, additional, Miller, Edward J., additional, Moon, James C., additional, Murthy, Venkatesh L., additional, Quarta, C. Cristina, additional, Rapezzi, Claudio, additional, Ruberg, Frederick L., additional, Shah, Sanjiv J., additional, Slart, Riemer H. J. A., additional, Verberne, Hein J., additional, and Bourque, Jamieson M., additional

Published
2021
Full Text
View/download PDF

59. Sex-Related Risk of Cardiac Involvement in Hereditary Transthyretin Amyloidosis : Insights From THAOS

Author

Caponetti, Angelo Giuseppe, Rapezzi, Claudio, Gagliardi, Christian, Milandri, Agnese, Dispenzieri, Angela, Kristen, Arnt V., Wixner, Jonas, Maurer, Mathew S., Garcia-Pavia, Pablo, Tournev, Ivailo, Planté-Bordeneuve, Violaine, Chapman, Douglass, and Amass, Leslie

Subjects
myocardial involvement, Kardiologi, sex, Cardiac and Cardiovascular Systems, registry, ATTRv amyloidosis
Abstract

Objectives: Because patients with ATTRv cardiomyopathy are more likely to be male, this analysis aimed to increase information on associations between sex and genotype, phenotype, and degree of myocardial involvement in ATTRv amyloidosis. Background: Transthyretin amyloid cardiomyopathy is a progressive, fatal disease that occurs due to accumulation of wild-type or variant (ATTRv) transthyretin amyloid fibrils in the myocardium. Methods: The Transthyretin Amyloidosis Outcomes Survey (THAOS) is an ongoing global longitudinal observational survey of patients with ATTR amyloidosis and asymptomatic carriers with TTR mutations. Data from THAOS (data cutoff: January 6, 2020) were analyzed to determine any sex-based differences in genotype, phenotype, and presence of cardiac and neurological symptoms in patients with ATTRv amyloidosis and in patients with ATTRv amyloidosis and cardiomyopathy. Results: There were 2,790 patients with ATTRv amyloidosis enrolled in THAOS, with male patients more likely to have symptoms of cardiac involvement and a cardiac phenotype. Male prevalence was greater in patients with more severe cardiac manifestations of disease, as assessed with N-terminal pro–B-type natriuretic peptide, left-ventricular (LV) ejection fraction, mean LV wall thickness divided by height, and LV mass index divided by height. Sex, age at disease onset, and genotype category were identified by multivariate analyses as risk factors for the development of cardiomyopathy (defined as increased LV septum thickness divided by height). Conclusions: In this analysis, myocardial involvement was more frequent and pronounced in male patients with ATTRv amyloidosis, suggesting that there may be biological characteristics that inhibit myocardial amyloid infiltration in females or facilitate it in males.

Published
2021

60. 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, Polydefkis, Michael, Gonzalez-Duarte, Alejandra, Wixner, Jonas, Kristen, Arnt, V, Schmidt, Hartmut H., Berk, John L., Losada Lopez, Ines Asuncion, Dispenzieri, Angela, Quan, Dianna, Conceicao, Isabel M., Slama, Michel S., Gillmore, Julian D., Kyriakides, Theodoros, Ajroud-Driss, Senda, Waddington-Cruz, Marcia, Mezei, Michelle M., Plante-Bordeneuve, Violaine, Attarian, Shahram, Mauricio, Elizabeth, Brannagan, Thomas H., III, Ueda, Mitsuharu, Aldinc, Emre, Wang, Jing Jing, White, Matthew T., Vest, John, Berber, Erhan, Sweetser, Marianne T., Coelho, Teresa, Adams, David, Polydefkis, Michael, Gonzalez-Duarte, Alejandra, Wixner, Jonas, Kristen, Arnt, V, Schmidt, Hartmut H., Berk, John L., Losada Lopez, Ines Asuncion, Dispenzieri, Angela, Quan, Dianna, Conceicao, Isabel M., Slama, Michel S., Gillmore, Julian D., Kyriakides, Theodoros, Ajroud-Driss, Senda, Waddington-Cruz, Marcia, Mezei, Michelle M., Plante-Bordeneuve, Violaine, Attarian, Shahram, Mauricio, Elizabeth, Brannagan, Thomas H., III, Ueda, Mitsuharu, Aldinc, Emre, Wang, Jing Jing, White, Matthew T., Vest, John, Berber, Erhan, Sweetser, Marianne T., and Coelho, Teresa

Abstract

Background Hereditary transthyretin-mediated amyloidosis is a rare, inherited, progressive disease caused by mutations in the transthyretin (TTR) gene. We assessed the safety and efficacy of long-term treatment with patisiran, an RNA interference therapeutic that inhibits TTR production, in patients with hereditary transthyretin-mediated amyloidosis with polyneuropathy. Methods This multicentre, open-label extension (OLE) trial enrolled patients at 43 hospitals or clinical centres in 19 countries as of Sept 24, 2018. Patients were eligible if they had completed the phase 3 APOLLO or phase 2 OLE parent studies and tolerated the study drug. Eligible patients from APOLLO (patisiran and placebo groups) and the phase 2 OLE (patisiran group) studies enrolled in this global OLE trial and received patisiran 0.3 mg/kg by intravenous infusion every 3 weeks with plans to continue to do so for up to 5 years. Efficacy assessments included measures of polyneuropathy (modified Neuropathy Impairment Score +7 [mNIS+7]), quality of life, autonomic symptoms, nutritional status, disability, ambulation status, motor function, and cardiac stress, with analysis by study groups (APOLLO-placebo, APOLLO-patisiran, phase 2 OLE patisiran) based on allocation in the parent trial. The global OLE is ongoing with no new enrolment, and current findings are based on the interim analysis of the patients who had completed 12-month efficacy assessments as of the data cutoff. Safety analyses included all patients who received one or more dose of patisiran up to the data cutoff. This study is registered with ClinicalTrials.gov, NCT02510261. Findings Between July 13, 2015, and Aug 21, 2017, of 212 eligible patients, 211 were enrolled: 137 patients from the APOLLO-patisiran group, 49 from the APOLLO-placebo group, and 25 from the phase 2 OLE patisiran group. At the data cutoff on Sept 24, 2018, 126 (92%) of 137 patients from the APOLLO-patisiran group, 38 (78%) of 49 from the APOLLO-placebo group, and 25 (

Published
2021
Full Text
View/download PDF

64. Amyloid in endomyocardial biopsies

Author

Kieninger, Barbara, Eriksson, Magdalena, Kandolf, Reinhard, Schnabel, Philipp A., Schönland, Stefan, Kristen, Arnt V., Hegenbart, Ute, Lohse, Peter, and Röcken, Christoph

Published
2010
Full Text
View/download PDF

70. Diagnosis and treatment of cardiac amyloidosis: a position statement of the ESC Working Group on Myocardial and Pericardial Diseases

Author

Garcia-Pavia, Pablo, primary, Rapezzi, Claudio, additional, Adler, Yehuda, additional, Arad, Michael, additional, Basso, Cristina, additional, Brucato, Antonio, additional, Burazor, Ivana, additional, Caforio, Alida L P, additional, Damy, Thibaud, additional, Eriksson, Urs, additional, Fontana, Marianna, additional, Gillmore, Julian D, additional, Gonzalez-Lopez, Esther, additional, Grogan, Martha, additional, Heymans, Stephane, additional, Imazio, Massimo, additional, Kindermann, Ingrid, additional, Kristen, Arnt V, additional, Maurer, Mathew S, additional, Merlini, Giampaolo, additional, Pantazis, Antonis, additional, Pankuweit, Sabine, additional, Rigopoulos, Angelos G, additional, and Linhart, Ales, additional

Published
2021
Full Text
View/download PDF

71. Diagnosis and treatment of cardiac amyloidosis. A position statement of the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases

Author

Garcia‐Pavia, Pablo, primary, Rapezzi, Claudio, additional, Adler, Yehuda, additional, Arad, Michael, additional, Basso, Cristina, additional, Brucato, Antonio, additional, Burazor, Ivana, additional, Caforio, Alida L.P., additional, Damy, Thibaud, additional, Eriksson, Urs, additional, Fontana, Marianna, additional, Gillmore, Julian D., additional, Gonzalez‐Lopez, Esther, additional, Grogan, Martha, additional, Heymans, Stephane, additional, Imazio, Massimo, additional, Kindermann, Ingrid, additional, Kristen, Arnt V., additional, Maurer, Mathew S., additional, Merlini, Giampaolo, additional, Pantazis, Antonis, additional, Pankuweit, Sabine, additional, Rigopoulos, Angelos G., additional, and Linhart, Ales, additional

Published
2021
Full Text
View/download PDF

74. Identifying Mixed Phenotype : Evaluating the Presence of Polyneuropathy in Patients with Hereditary Transthyretin-Mediated Amyloidosis with Cardiomyopathy

Author

Merkel, Madeline, Grogan, Martha, Hawkins, Philip N., Kristen, Arnt V., Berk, John L., Suhr, Ole B., Lin, Hollis, McManus, Anastasia, Powell, Christine, Vest, John, Karsten, Verena, Judge, Daniel P., Merkel, Madeline, Grogan, Martha, Hawkins, Philip N., Kristen, Arnt V., Berk, John L., Suhr, Ole B., Lin, Hollis, McManus, Anastasia, Powell, Christine, Vest, John, Karsten, Verena, and Judge, Daniel P.

Abstract

Supplement: SMeeting Abstract: 240

Published
2020

75. 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
Full Text
View/download PDF

76. 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
Full Text
View/download PDF

78. Identifying Mixed Phenotype: Evaluating the Presence of Polyneuropathy in Patients with Hereditary Transthyretin-Mediated Amyloidosis with Cardiomyopathy (240)

Author

Merkel, Madeline, primary, Grogan, Martha, additional, Hawkins, Philip N., additional, Kristen, Arnt V., additional, Berk, John L., additional, Suhr, Ole B., additional, Lin, Hollis, additional, McManus, Anastasia, additional, Powell, Christine, additional, Vest, John, additional, Karsten, Verena, additional, and Judge, Daniel P., additional

Published
2020
Full Text
View/download PDF

79. Quality of life outcomes in APOLLO, the phase 3 trial of the RNAi therapeutic patisiran in patients with hereditary transthyretin-mediated amyloidosis

Author

Obici, Laura, primary, Berk, John L., additional, González-Duarte, Alejandra, additional, Coelho, Teresa, additional, Gillmore, Julian, additional, Schmidt, Hartmut H.-J., additional, Schilling, Matthias, additional, Yamashita, Taro, additional, Labeyrie, Céline, additional, Brannagan, Thomas H., additional, Ajroud-Driss, Senda, additional, Gorevic, Peter, additional, Kristen, Arnt V., additional, Franklin, Jaclyn, additional, Chen, Jihong, additional, Sweetser, Marianne T., additional, Wang, Jing Jing, additional, and Adams, David, additional

Published
2020
Full Text
View/download PDF

82. Transthyretin cardiac amyloidosis in continental Western Europe: an insight through the Transthyretin Amyloidosis Outcomes Survey (THAOS).

Author

Damy, Thibaud, Kristen, Arnt V, Suhr, Ole B, Maurer, Mathew S, Planté-Bordeneuve, Violaine, Yu, Ching-Ray, Ong, Moh-Lim, Coelho, Teresa, Rapezzi, Claudio, and Investigators, THAOS

Subjects
TRANSTHYRETIN, AMYLOIDOSIS, GENOTYPES, CLINICAL trials, PHENOTYPES
Abstract

Aims Transthyretin amyloidosis (ATTR amyloidosis) is a heterogeneous disorder with cardiac, neurologic, and mixed phenotypes. We describe the phenotypic and genotypic profiles of this disease in continental Western Europe as it appears from the Transthyretin Amyloidosis Survey (THAOS). Methods and results THAOS is an ongoing, worldwide, longitudinal, observational survey established to study differences in presentation, diagnosis, and natural history in ATTR amyloidosis subjects. At data cut-off, 1411 symptomatic subjects from nine continental Western European countries were enrolled in THAOS [1286 hereditary (ATTRm) amyloidosis; 125 wild-type ATTR (ATTRwt) amyloidosis]. Genotypes and phenotypes varied notably by country. Four mutations (Val122Ile, Leu111Met, Thr60Ala, and Ile68Leu), and ATTRwt, were associated with a mainly cardiac phenotype showing symmetric left ventricular (LV) hypertrophy, normal diastolic LV dimensions and volume, and mildly depressed LV ejection fraction (LVEF). Morphologic and functional abnormalities on echocardiogram were significantly more severe in subjects with cardiac (n '= 210), compared with a mixed (n = 298), phenotype: higher median (Q1–Q3) interventricular septal thickness [18 (16–21) vs. 16 (13–20) mm; P = 0.0006]; and more frequent incidence of LVEF <50% (38.1 vs. 17.5%; P = 0.0008). Subjects with cardiac mutations or ATTRwt (or cardiac or mixed phenotype) had a lower survival rate than subjects in other genotype (or the neurologic phenotype) categories (P < 0.0001, for both). Conclusion ATTR amyloidosis genotypes and phenotypes are highly heterogeneous in continental Western Europe. A geographic map of the different disease profiles and awareness that a subset of subjects have a dominant cardiac phenotype, mimicking hypertrophic cardiomyopathy, at presentation can facilitate the clinical recognition of this underdiagnosed disease. Trial registration ClinicalTrials.gov: NCT00628745. [ABSTRACT FROM AUTHOR]

Published
2022
Full Text
View/download PDF

83. Expert Consensus Recommendations for the Suspicion and Diagnosis of Cardiac ATTR Amyloidosis

Author

Maurer, Mathew S., Bokhari, Sabahat, Damy, Thibaud, Dorbala, Sharmila, Drachman, Brian M., Fontana, Marianna, Grogan, Martha, Kristen, Arnt V., Lousada, Isabelle, Nativi-Nicolau, Jose, Quarta, Candida Cristina, Rapezzi, Claudio, Ruberg, Frederick L., Witteles, Ronald, and Merlini, Giampaolo

Subjects
Amyloid Neuropathies, Familial, Consensus, Practice Guidelines as Topic, Humans, Cardiomyopathies, Article
Abstract

Cardiomyopathy is a manifestation of transthyretin amyloidosis (ATTR), which is an underrecognized systemic disease whereby the transthyretin protein misfolds to form fibrils that deposit in various tissues and organs. ATTR amyloidosis is debilitating and associated with poor life expectancy, especially in those with cardiac dysfunction, but a variety of treatment options have recently become available. Considered a rare disease, ATTR amyloidosis may be more prevalent than thought, particularly in older persons. Diagnosis is often delayed because of a lack of disease awareness and the heterogeneity of symptoms at presentation. Given the recent availability of effective treatments, early recognition and diagnosis are especially critical because treatment is likely more effective earlier in the disease course. The Amyloidosis Research Consortium recently convened a group of experts in ATTR amyloidosis who, through an iterative process, agreed on best practices for suspicion, diagnosis, and characterization of disease. This review describes these consensus recommendations for ATTR associated with cardiomyopathy as a resource to aid cardiologists and others in the recognition and diagnosis of ATTR associated with cardiomyopathy. Included in this review is an overview of red flag signs and symptoms and a recommended diagnostic approach, including testing for monoclonal protein, scintigraphy, or biopsy and, if ATTR associated with cardiomyopathy is identified, TTR genotyping.

Published
2019

85. Identifying Mixed Phenotype : Evaluating the Presence of Polyneuropathy in Patients with Hereditary Transthyretin-Mediated Amyloidosis with Cardiomyopathy

Author

Grogan, Martha, Hawkins, Philip N., Kristen, Arnt V., Berk, John L., Suhr, Ole B., Lin, Hollis, Merkel, Madeline, McManus, Anastasia, Powell, Christine, Vest, John, Karsten, Verena, Judge, Daniel P., Grogan, Martha, Hawkins, Philip N., Kristen, Arnt V., Berk, John L., Suhr, Ole B., Lin, Hollis, Merkel, Madeline, McManus, Anastasia, Powell, Christine, Vest, John, Karsten, Verena, and Judge, Daniel P.

Published
2019
Full Text
View/download PDF

86. ASNC/AHA/ASE/EANM/HFSA/ISA/SCMR/SNMMI expert consensus recommendations for multimodality imaging in cardiac amyloidosis: Part 1 of 2-evidence base and standardized methods of imaging.

Author

UCL - (SLuc) Centre du cancer, UCL - SSS/IREC/SLUC - Pôle St.-Luc, UCL - (SLuc) Service de médecine nucléaire, Dorbala, Sharmila, Ando, Yukio, Bokhari, Sabahat, Dispenzieri, Angela, Falk, Rodney H, Ferrari, Victor A, Fontana, Marianna, Gheysens, Olivier, Gillmore, Julian D, Glaudemans, Andor W J M, Hanna, Mazen A, Hazenberg, Bouke P C, Kristen, Arnt V, Kwong, Raymond Y, Maurer, Mathew S, Merlini, Giampaolo, Miller, Edward J, Moon, James C, Murthy, Venkatesh L, Quarta, C Cristina, Rapezzi, Claudio, Ruberg, Frederick L, Shah, Sanjiv J, Slart, Riemer H J A, Verberne, Hein J, Bourque, Jamieson M, UCL - (SLuc) Centre du cancer, UCL - SSS/IREC/SLUC - Pôle St.-Luc, UCL - (SLuc) Service de médecine nucléaire, Dorbala, Sharmila, Ando, Yukio, Bokhari, Sabahat, Dispenzieri, Angela, Falk, Rodney H, Ferrari, Victor A, Fontana, Marianna, Gheysens, Olivier, Gillmore, Julian D, Glaudemans, Andor W J M, Hanna, Mazen A, Hazenberg, Bouke P C, Kristen, Arnt V, Kwong, Raymond Y, Maurer, Mathew S, Merlini, Giampaolo, Miller, Edward J, Moon, James C, Murthy, Venkatesh L, Quarta, C Cristina, Rapezzi, Claudio, Ruberg, Frederick L, Shah, Sanjiv J, Slart, Riemer H J A, Verberne, Hein J, and Bourque, Jamieson M

Abstract

No abstract available

Published
2019

87. Transthyretin cardiac amyloidosis in continental Western Europe : An insight through the Transthyretin Amyloidosis Outcomes Survey (THAOS)

Author

Damy, Thibaud, Kristen, Arnt V, Suhr, Ole B., Maurer, Mathew S, Planté-Bordeneuve, Violaine, Yu, Ching-Ray, Ong, Moh-Lim, Coelho, Teresa, Rapezzi, Claudio, Damy, Thibaud, Kristen, Arnt V, Suhr, Ole B., Maurer, Mathew S, Planté-Bordeneuve, Violaine, Yu, Ching-Ray, Ong, Moh-Lim, Coelho, Teresa, and Rapezzi, Claudio

Abstract

Aims: Transthyretin amyloidosis (ATTR amyloidosis) is a heterogeneous disorder with cardiac, neurologic, and mixed phenotypes. We describe the phenotypic and genotypic profiles of this disease in continental Western Europe as it appears from the Transthyretin Amyloidosis Survey (THAOS). Methods and results: THAOS is an ongoing, worldwide, longitudinal, observational survey established to study differences in presentation, diagnosis, and natural history in ATTR amyloidosis subjects. At data cut-off, 1411 symptomatic subjects from nine continental Western European countries were enrolled in THAOS [1286 hereditary (ATTRm) amyloidosis; 125 wild-type ATTR (ATTRwt) amyloidosis]. Genotypes and phenotypes varied notably by country. Four mutations (Val122Ile, Leu111Met, Thr60Ala, and Ile68Leu), and ATTRwt, were associated with a mainly cardiac phenotype showing symmetric left ventricular (LV) hypertrophy, normal diastolic LV dimensions and volume, and mildly depressed LV ejection fraction (LVEF). Morphologic and functional abnormalities on echocardiogram were significantly more severe in subjects with cardiac (n = 210), compared with a mixed (n = 298), phenotype: higher median (Q1-Q3) interventricular septal thickness [18 (16-21) vs. 16 (13-20) mm; P = 0.0006]; and more frequent incidence of LVEF <50% (38.1 vs. 17.5%; P = 0.0008). Subjects with cardiac mutations or ATTRwt (or cardiac or mixed phenotype) had a lower survival rate than subjects in other genotype (or the neurologic phenotype) categories (P < 0.0001, for both). Conclusion: ATTR amyloidosis genotypes and phenotypes are highly heterogeneous in continental Western Europe. A geographic map of the different disease profiles and awareness that a subset of subjects have a dominant cardiac phenotype, mimicking hypertrophic cardiomyopathy, at presentation can facilitate the clinical recognition of this underdiagnosed disease. Trial registration: ClinicalTrials.gov: NCT00628745.

Published
2019
Full Text
View/download PDF

89. Impact of genotype and phenotype on cardiac biomarkers in patients with transthyretin amyloidosis - Report from the Transthyretin Amyloidosis Outcome Survey (THAOS)

Author

Kristen, Arnt V., Maurer, Mathew S., Rapezzi, Claudio, Mundayat, Rajiv, Suhr, Ole B., Damy, Thibaud, Barroso, Fabio Adrian, Rugiero, Marcelo F, Van Cleemput, Johan J., Tournev, Ivailo, Cruz, Marcia Waddington, Fine, Nowell M., Kristen, Arnt Volko, Schmidt, Hartmut H. J., Zimmermann, Tim, Gess, Burkhard, Moelgaard, Henning, Plana, Josep Maria Campistol, Reines, Juan Buades, Costello, Jose Gonzalez, Pavia, Pablo Garcia, Blanco, Jose Luis Munoz, Plante Bordeneuve, Violaine, Adams, David, Inamo, Jocelyn, Vita, Giuseppe, Merlini, Giampaolo, Bergesio, Franco, Sekijima, Yoshiki, Ando, Yukio, Misawa, Sonoko, Lee, Ga Yeon, Jeeyoung, Oh, Briseno, Maria Alejandra Gonzalez Duarte, Hazenberg, Bouke P. C., Coelho, Teresa, Conceicao, Isabel M., Maurer, Mathew Shane, Shah, Sanjiv Jayendra, Quan, Dianna, Judge, Daniel Philip, Gottlieb, Stephen Scott, Sarswat, Nitasha, Murali, Srinivas C., Iyadurai, Stanley, Cotts, William Gerritt, Drachman, Brian M., Dispenzieri, Angela, Steidley, David Eric, Hummel, Scott L., Lenihan, Daniel J., Ventura, Hector Osvaldo, Jacoby, Daniel L., Hoffman, James E., Kristen, Arnt V., Maurer, Mathew S., Rapezzi, Claudio, Mundayat, Rajiv, Suhr, Ole B., Damy, Thibaud, Barroso, Fabio Adrian, Rugiero, Marcelo F, Van Cleemput, Johan J., Tournev, Ivailo, Cruz, Marcia Waddington, Fine, Nowell M., Kristen, Arnt Volko, Schmidt, Hartmut H.J., Zimmermann, Tim, Gess, Burkhard, Moelgaard, Henning, Plana, Josep Maria Campistol, Reines, Juan Buade, Costello, Jose Gonzalez, Pavia, Pablo Garcia, Blanco, Jose Luis Munoz, Plante-Bordeneuve, Violaine, Adams, David, Inamo, Jocelyn, Vita, Giuseppe, Merlini, Giampaolo, Bergesio, Franco, Sekijima, Yoshiki, Ando, Yukio, Misawa, Sonoko, Lee, Ga Yeon, Oh, Jeeyoung, Briseno, Maria Alejandra Gonzalez Duarte, Hazenberg, Bouke P.C., Coelho, Teresa, Conceicao, Isabel M., Maurer, Mathew Shane, Shah, Sanjiv Jayendra, Quan, Dianna, Judge, Daniel Philip, Gottlieb, Stephen Scott, Sarswat, Nitasha, Murali, Srinivas C., Iyadurai, Stanley, Cotts, William Gerritt, Drachman, Brian M., Dispenzieri, Angela, Steidley, David Eric, Hummel, Scott L., Lenihan, Daniel J., Ventura, Hector Osvaldo, Jacoby, Daniel L., Hoffman, James E., and Translational Immunology Groningen (TRIGR)

Subjects
Genetics and Molecular Biology (all), Pathology, Physiology, medicine.medical_treatment, Peptide Hormones, lcsh:Medicine, Medicine (all), Biochemistry, Agricultural and Biological Sciences (all), 030204 cardiovascular system & hematology, Liver transplantation, Pathology and Laboratory Medicine, Body Mass Index, 0302 clinical medicine, Genotype-phenotype distinction, Surveys and Questionnaires, Genotype, Natriuretic Peptide, Brain, Natriuretic peptide, Medicine and Health Sciences, Atrial natriuretic peptides, Cardiac and Cardiovascular Systems, lcsh:Science, Multidisciplinary, Kardiologi, biology, Amyloidosis, Biochemical markers, Troponin, Phenotype, Physiological Parameters, cardiovascular system, Anatomy, hormones, hormone substitutes, and hormone antagonists, Research Article, medicine.medical_specialty, medicine.drug_class, Cardiology, Surgical and Invasive Medical Procedures, macromolecular substances, NO, 03 medical and health sciences, Digestive System Procedures, Signs and Symptoms, Troponin T, Diagnostic Medicine, Natriuretic Peptide, medicine, Humans, cardiovascular diseases, Heart Failure, Amyloid Neuropathies, Familial, Transplantation, Biochemistry, Genetics and Molecular Biology (all), business.industry, lcsh:R, Troponin I, Body Weight, Biology and Life Sciences, Proteins, Renal System, Organ Transplantation, medicine.disease, Hormones, Liver Transplantation, Transthyretin, Cytoskeletal Proteins, Heart failure, biology.protein, lcsh:Q, business, 030217 neurology & neurosurgery, Biomarkers
Abstract

AIM:Cardiac troponins and natriuretic peptides are established for risk stratification in light-chain amyloidosis. Data on cardiac biomarkers in transthyretin amyloidosis (ATTR) are lacking. METHODS AND RESULTS:Patients (n = 1617) with any of the following cardiac biomarkers, BNP (n = 1079), NT-proBNP (n = 550), troponin T (n = 274), and troponin I (n = 108), available at baseline in the Transthyretin Amyloidosis Outcomes Survey (THAOS) were analyzed for differences between genotypes and phenotypes and their association with survival. Median level of BNP was 68.0 pg/mL (IQR 30.5-194.9), NT-proBNP 337.9 pg/mL (IQR 73.0-2584.0), troponin T 0.03 μg/L (IQR 0.01-0.05), and troponin I 0.08 μg/L (IQR 0.04-0.13). NT-proBNP and BNP were higher in wild-type than mutant-type ATTR, troponin T and I did not differ, respectively. Non-Val30Met patients had higher BNP, NT-proBNP and troponin T levels than Val30Met patients, but not troponin I. Late-onset Val30Met was associated with higher levels of troponin I and troponin T compared with early-onset. 115 patients died during a median follow-up of 1.2 years. Mortality increased with increasing quartiles (BNP/NT-proBNP Q1 = 1.7%, Q2 = 5.2%, Q3 = 21.7%, Q4 = 71.3%; troponin T/I Q1 = 6.5%, Q2 = 14.5%, Q3 = 33.9%, Q4 = 45.2%). Three-year overall-survival estimates for BNP/NT-proBNP and troponin T/I quartiles differed significantly (p

Published
2017

90. ASNC/AHA/ASE/EANM/HFSA/ISA/SCMR/ SNMMI Expert Consensus Recommendations for Multimodality Imaging in Cardiac Amyloidosis: Part 2 of 2—Diagnostic Criteria and Appropriate Utilization.

Author

Dorbala, Sharmila, Yukio Ando, Bokhari, Sabahat, Dispenzieri, Angela, Falk, Rodney H., Ferrari, Victor A., Fontana, Marianna, Gheysens, Olivier, Gillmore, Julian D., Glaudemans, Andor W. J. M., Hanna, Mazen A., Hazenberg, Bouke P. C., Kristen, Arnt V., Kwong, Raymond Y., Maurer, Mathew S., Merlini, Giampaolo, Miller, Edward J., Moon, James C., Murthy, Venkatesh L., and Quarta, C. Cristina

Published
2021
Full Text
View/download PDF

91. ASNC/AHA/ASE/EANM/HFSA/ISA/SCMR/ SNMMI Expert Consensus Recommendations for Multimodality Imaging in Cardiac Amyloidosis: Part 1 of 2—Evidence Base and Standardized Methods of Imaging.

Author

Dorbala, Sharmila, Yukio Ando, Bokhari, Sabahat, Dispenzieri, Angela, Falk, Rodney H., Ferrari, Victor A., Fontana, Marianna, Gheysens, Olivier, Gillmore, Julian D., Glaudemans, Andor W. J. M., Hanna, Mazen A., Hazenberg, Bouke P. C., Kristen, Arnt V., Kwong, Raymond Y., Maurer, Mathew S., Merlini, Giampaolo, Miller, Edward J., Moon, James C., Murthy, Venkatesh L., and Quarta, C. Cristina

Published
2021
Full Text
View/download PDF

92. 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
Full Text
View/download PDF

93. Analysis of autonomic outcomes in APOLLO, a phase III trial of the RNAi therapeutic patisiran in patients with hereditary transthyretin-mediated amyloidosis

Author

González-Duarte, Alejandra, primary, Berk, John L., additional, Quan, Dianna, additional, Mauermann, Michelle L., additional, Schmidt, Hartmut H., additional, Polydefkis, Michael, additional, Waddington-Cruz, Márcia, additional, Ueda, Mitsuharu, additional, Conceição, Isabel M., additional, Kristen, Arnt V., additional, Coelho, Teresa, additional, Cauquil, Cécile A., additional, Tard, Céline, additional, Merkel, Madeline, additional, Aldinc, Emre, additional, Chen, Jihong, additional, Sweetser, Marianne T., additional, Wang, Jing Jing, additional, and Adams, David, additional

Published
2019
Full Text
View/download PDF

94. ASNC/AHA/ASE/EANM/HFSA/ISA/SCMR/SNMMI Expert Consensus Recommendations for Multimodality Imaging in Cardiac Amyloidosis: Part 1 of 2—Evidence Base and Standardized Methods of Imaging

Author

Dorbala, Sharmila, primary, Ando, Yukio, additional, Bokhari, Sabahat, additional, Dispenzieri, Angela, additional, Falk, Rodney H., additional, Ferrari, Victor A., additional, Fontana, Marianna, additional, Gheysens, Olivier, additional, Gillmore, Julian D., additional, Glaudemans, Andor W.J.M., additional, Hanna, Mazen A., additional, Hazenberg, Bouke P.C., additional, Kristen, Arnt V., additional, Kwong, Raymond Y., additional, Maurer, Mathew S., additional, Merlini, Giampaolo, additional, Miller, Edward J., additional, Moon, James C., additional, Murthy, Venkatesh L., additional, Quarta, C. Cristina, additional, Rapezzi, Claudio, additional, Ruberg, Frederick L., additional, Shah, Sanjiv J., additional, Slart, Riemer H.J.A., additional, Verberne, Hein J., additional, and Bourque, Jamieson M., additional

Published
2019
Full Text
View/download PDF

95. ASNC/AHA/ASE/EANM/HFSA/ISA/SCMR/SNMMI expert consensus recommendations for multimodality imaging in cardiac amyloidosis: Part 2 of 2—Diagnostic criteria and appropriate utilization

Author

Dorbala, Sharmila, primary, Ando, Yukio, additional, Bokhari, Sabahat, additional, Dispenzieri, Angela, additional, Falk, Rodney H., additional, Ferrari, Victor A., additional, Fontana, Marianna, additional, Gheysens, Olivier, additional, Gillmore, Julian D., additional, Glaudemans, Andor W. J. M., additional, Hanna, Mazen A., additional, Hazenberg, Bouke P. C., additional, Kristen, Arnt V., additional, Kwong, Raymond Y., additional, Maurer, Mathew S., additional, Merlini, Giampaolo, additional, Miller, Edward J., additional, Moon, James C., additional, Murthy, Venkatesh L., additional, Quarta, C. Cristina, additional, Rapezzi, Claudio, additional, Ruberg, Frederick L., additional, Shah, Sanjiv J., additional, Slart, Riemer H. J. A., additional, Verberne, Hein J., additional, and Bourque, Jamieson M., additional

Published
2019
Full Text
View/download PDF

96. Identifying Mixed Phenotype: Evaluating the Presence of Polyneuropathy in Patients with Hereditary Transthyretin-Mediated Amyloidosis with Cardiomyopathy

Author

Grogan, Martha, primary, Hawkins, Philip N., additional, Kristen, Arnt V., additional, Berk, John L., additional, Suhr, Ole B., additional, Lin, Hollis, additional, Merkel, Madeline, additional, McManus, Anastasia, additional, Powell, Christine, additional, Vest, John, additional, Karsten, Verena, additional, and Judge, Daniel P., additional

Published
2019
Full Text
View/download PDF

97. Association of Patisiran, an RNA Interference Therapeutic, With Regional Left Ventricular Myocardial Strain in Hereditary Transthyretin Amyloidosis

Author

Minamisawa, Masatoshi, primary, Claggett, Brian, additional, Adams, David, additional, Kristen, Arnt V., additional, Merlini, Giampaolo, additional, Slama, Michel S., additional, Dispenzieri, Angela, additional, Shah, Amil M., additional, Falk, Rodney H., additional, Karsten, Verena, additional, Sweetser, Marianne T., additional, Chen, Jihong, additional, Riese, Richard, additional, Vest, John, additional, and Solomon, Scott D., additional

Published
2019
Full Text
View/download PDF

99. Performance analysis of AL amyloidosis cardiac biomarker staging systems with special focus on renal failure and atrial arrhythmia

Author

Dittrich, Tobias, primary, Benner, Axel, additional, Kimmich, Christoph, additional, Siepen, Fabian aus dem, additional, Veelken, Kaya, additional, Kristen, Arnt V., additional, Bochtler, Tilmann, additional, Katus, Hugo A., additional, Müller-Tidow, Carsten, additional, Hegenbart, Ute, additional, and Schönland, Stefan O., additional

Published
2019
Full Text
View/download PDF

100. Efficacy of Tafamidis in Transthyretin Amyloid Cardiomyopathy in the ATTR-ACT Trial: Sensitivity Analyses Further Support the Primary Results

Author

Maurer, Mathew S., primary, Schwartz, Jeffrey H., additional, Gundapaneni, Balarama, additional, Elliott, Perry, additional, Merlini, Giampaolo, additional, Cruz, Márcia Waddington, additional, Kristen, Arnt V., additional, Grogan, Martha, additional, Witteles, Ronald, additional, Damy, Thibaud, additional, Drachman, Brian M., additional, Shah, Sanjiv J., additional, Hanna, Mazen, additional, Judge, Daniel P., additional, Gottlieb, Stephen S., additional, Berk, John L., additional, Lenihan, Daniel J., additional, Hoffman, James E., additional, Hummel, Scott L., additional, Velazquez, Eric J., additional, Patterson, Terrell A., additional, Sultan, Marla B., additional, and Rapezzi, Claudio, additional

Published
2018
Full Text
View/download PDF

Catalog

Books, media, physical & digital resources
See catalog results