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1. Effect of Acoramidis on Myocardial Structure and Function in Transthyretin Amyloid Cardiomyopathy: Insights From the ATTRibute-CM Cardiac Magnetic Resonance (CMR) Substudy

Author

Razvi, Yousuf, Judge, Daniel P., Martinez-Naharro, Ana, Ioannou, Adam, Venneri, Lucia, Patel, Rishi, Gillmore, Julian D., Kellman, Peter, Edwards, Laura, Taubel, Jorg, Du, Jing, Tamby, Jean-François, Castaño, Adam, Siddhanti, Suresh, Katz, Leonid, Fox, Jonathan C., and Fontana, Marianna

Published
2024
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2. Abstract 4144740: Characteristics and Natural History of Early ATTR Cardiac Amyloid Infiltration

Author

Porcari, Aldostefano, Razvi, Yousuf, Cappelli, Francesco, Nitsche, Christian, Serenelli, Matteo, Longhi, Simone, Sinigiani, Giulio, Cipriani, Alberto, Aimo, Alberto, Allegro, Valentina, Tomasoni, Daniela, zampieri, Mattia, Cantone, Anna, Masri, Ahmad, Urey, Marcus, Ioannou, Adam, Petrie, Aviva, Gustafsson, Finn, Whelan, Carol, Emdin, Michele, Metra, Marco, sinagra, Gianfranco, Lachmann, Helen, Wechalekar, Ashutosh, Hawkins, Philip, Gillmore, Julian, and Fontana, Marianna

Published
2024
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8. Tracking Multiorgan Treatment Response in Systemic AL-Amyloidosis With Cardiac Magnetic Resonance Derived Extracellular Volume Mapping

Author

Ioannou, Adam, Patel, Rishi K., Martinez-Naharro, Ana, Razvi, Yousuf, Porcari, Aldostefano, Hutt, David F., Bandera, Francesco, Kotecha, Tushar, Venneri, Lucia, Chacko, Liza, Massa, Paolo, Hanger, Melissa, Knight, Daniel, Manisty, Charlotte, Moon, James, Quarta, Cristina, Lachmann, Helen, Whelan, Carol, Kellman, Peter, Hawkins, Philip N., Gillmore, Julian D., Wechelakar, Ashutosh, and Fontana, Marianna

Published
2023
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9. Abstract 11740: Redefining Cardiac Involvement in Systemic Immunoglobulin Light Chain Amyloidosis and Treatment Implications

Author

Porcari, Aldostefano, Masi, Ambra, Martinez-Naharro, Ana, Razvi, Yousuf, Patel, Rishi, Ioannou, Adam, Rauf, Muhammad, Sinigiani, Giulio, Wisniowski, Brendan, Filisetti, Stefano, CURRIE-CATHEY, Jasmine, OʼBeara, Sophie, Kotecha, Tushar, Knight, Daniel, Moon, James, sinagra, Gianfranco, Virsinskaite, Ruta, GILBERTSON, Janet A, Venneri, Lucia, Petrie, Aviva, Lachmann, Helen, Whelan, Carol, Kellman, Peter, Ravichandran, Sriram, Cohen, Oliver, Mahmood, Shameem, Hawkins, Philip N, Gillmore, Julian D, Wechalekar, Ashutosh, and Fontana, Marianna

Published
2023
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14. Redefining Cardiac Involvement and Targets of Treatment in Systemic Immunoglobulin AL Amyloidosis.

Author

Porcari, Aldostefano, Masi, Ambra, Martinez-Naharro, Ana, Razvi, Yousuf, Patel, Rishi, Ioannou, Adam, Rauf, Muhammad U., Sinigiani, Giulio, Wisniowski, Brendan, Filisetti, Stefano, Currie-Cathey, Jasmine, O'Beara, Sophie, Kotecha, Tushar, Knight, Dan, Moon, James C., Sinagra, Gianfranco, Virsinskaite, Ruta, Gilbertson, Janet, Venneri, Lucia, and Petrie, Aviva

Published
2024
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15. Prognostic Value of a 6-Minute Walk Test in Patients With Transthyretin Cardiac Amyloidosis

Author

Ioannou, Adam, primary, Fumagalli, Carlo, additional, Razvi, Yousuf, additional, Porcari, Aldostefano, additional, Rauf, Muhammad U., additional, Martinez-Naharro, Ana, additional, Venneri, Lucia, additional, Moody, William, additional, Steeds, Richard P., additional, Petrie, Aviva, additional, Whelan, Carol, additional, Wechalekar, Ashutosh, additional, Lachmann, Helen, additional, Hawkins, Philip N., additional, Solomon, Scott D., additional, Gillmore, Julian D., additional, and Fontana, Marianna, additional

Published
2024
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16. Kidney Outcomes in Transthyretin Amyloid Cardiomyopathy

Author

Ioannou, Adam, Razvi, Yousuf, Porcari, Aldostefano, Rauf, Muhammad U., Martinez-Naharro, Ana, Venneri, Lucia, Kazi, Salsabeel, Pasyar, Ali, Luxhøj, Carina M., Petrie, Aviva, Moody, William, Steeds, Richard P., Sperry, Brett W., Witteles, Ronald M., Whelan, Carol, Wechalekar, Ashutosh, Lachmann, Helen, Hawkins, Philip N., Solomon, Scott D., Gillmore, Julian D., and Fontana, Marianna

Abstract

IMPORTANCE: Transthyretin amyloid cardiomyopathy (ATTR-CM) is a progressive cardiomyopathy that commonly presents with concomitant chronic kidney disease. Chronic kidney dysfunction is associated with worse outcomes, but the prognostic value of changes in kidney function over time has yet to be defined. OBJECTIVE: To assess the prognostic importance of a decline in estimated glomerular filtration rate (eGFR) in a large cohort of patients with ATTR-CM. DESIGN, SETTING, AND PARTICIPANTS: This retrospective, observational, single-center cohort study evaluated patients diagnosed with ATTR-CM at the National Amyloidosis Centre (NAC) in the UK who underwent an eGFR baseline assessment and a follow-up assessment at 1 year between January 2000 and April 2024. Data analysis was performed in June 2024. MAIN OUTCOMES AND MEASURES: The primary outcome was the risk of all-cause mortality associated with decline in kidney function (defined as a decrease in eGFR >20%). RESULTS: Among 2001 patients, mean (SD) age was 75.5 (8.4) years, and 263 patients (13.1%) were female. The median (IQR) change in eGFR was −5 mlL/min/1.73 m2 (−12 to 1), and 481 patients (24.0%) experienced decline in kidney function. Patients who experienced decline in kidney function more often had the p.(V142I) genotype than patients with stable kidney function (99 [20.6%] vs 202 [13.3%]; P < .001) and had a more severe cardiac phenotype at baseline, as evidenced by higher median (IQR) concentrations of serum cardiac biomarkers (N-terminal pro-B-type natriuretic peptide [NT-proBNP]: 2949 pg/mL [1759-5182] vs 2309 pg/mL [1146-4290]; P < .001; troponin T: 0.060 ng/mL [0.042-0.086] vs 0.052 ng/mL [0.033-0.074]; P < .001), while baseline median (IQR) kidney function was similar between the 2 groups (eGFR: 63 mL/min/1.73 m2 [51-77] vs 61 mL/min/1.73 m2 [49-77]; P = .41). Decline in kidney function was associated with a 1.7-fold higher risk of mortality (hazard ratio [HR], 1.71; 95% CI, 1.43-2.04; P < .001), with a similar risk across the 3 genotypes (wild type: HR, 1.64; 95% CI, 1.31-2.04; p.(V142I): HR, 1.70; 95% CI, 1.21-2.39; non-p.(V142I): HR, 1.51; 95% CI, 0.87-2.61) (P for interaction = .93) and the 3 NAC disease stages (stage 1: HR, 1.69; 95% CI, 1.22-2.32; stage 2: HR, 1.69; 95% CI, 1.30-2.18; stage 3: HR, 1.61; 95% CI, 1.11-2.35) (P for interaction = .97). Decline in kidney function remained independently associated with mortality after adjusting for increases in NT-proBNP and outpatient diuretic intensification (HR, 1.48; 95% CI, 1.23-2.76; P < .001). CONCLUSIONS AND RELEVANCE: In this retrospective cohort study, decline in kidney function was frequent in patients with ATTR-CM and was consistently associated with an increased risk of mortality, even after adjusting for established markers of worsening ATTR-CM. eGFR decline represents an independent marker of ATTR-CM disease progression that could guide treatment optimization in clinical practice.

Published
2025
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20. Microvascular obstruction in cardiac amyloidosis.

Author

Netti, Lucrezia, Ioannou, Adam, Martinez‐Naharro, Ana, Razvi, Yousuf, Porcari, Aldostefano, Venneri, Lucia, Maestrini, Viviana, Knight, Dan, Virsinskaite, Ruta, Rauf, Muhammad U., Kotecha, Tushar, Patel, Rishi K., Wechelakar, Ashutosh, Lachmann, Helen, Kellman, Peter, Manisty, Charlotte, Moon, James, Hawkins, Philip N., Gillmore, Julian D., and Fontana, Marianna

Subjects
*GLOBAL longitudinal strain, *CARDIAC magnetic resonance imaging, *PEPTIDES, *EXTRACELLULAR space, *CELL size, *CARDIAC amyloidosis
Abstract

Aims Methods and results Conclusions Cardiac amyloidosis (CA) is characterized by deposition of amyloid fibrils within the extracellular space, causing disarray of the myocardial structure and capillary architecture. This study aims to characterize the prevalence of microvascular obstruction (MVO) in patients with CA and to assess the association between MVO and prognosis.The study population comprised 800 patients, of which 400 had light‐chain CA (AL‐CA) and 400 had transthyretin CA (ATTR‐CA). MVO was present in 221 (27.6%) patients, and more common in ATTR‐CA than AL‐CA (124 [56.1%] vs. 97 [43.9%], p = 0.033). Patients with MVO had a more severe cardiac phenotype evidenced by higher N‐terminal pro‐brain natriuretic peptide (3516 ng/L [1944–6247] vs. 2508 ng/L [1203–5752], p < 0.001), worse global longitudinal strain (−10.5% [−12.6; −7.9] vs. −12.0% [−16.0; −8.9], p < 0.001), and higher extracellular volume (56% [51–61] vs. 50% [45–57], p < 0.001). Patients with AL‐CA and MVO had a higher serum troponin (86 ng/L [47–148] vs. 59 ng/L [44–78], p < 0.001), and higher T2 (53 ms [50–56] vs. 50 ms [48–52], p < 0.001), but lower extracellular volume (55% [50–60] vs. 58% [53–61], p = 0.008) and lower indexed myocyte cell volume (48.6 g/m2 [41.1–59.8] vs. 55.7 g/m2 [47.5–68.4], p < 0.001) than patients with ATTR‐CA and MVO. MVO was associated with an increased risk of mortality in the overall population (hazard ratio [HR] 1.28, 95% confidence interval [CI] 1.03–1.59, p = 0.025), and the subgroup with AL‐CA (HR 1.59, 95% CI 1.17–2.17, p = 0.003) but not ATTR‐CA (HR 1.04, 95% CI 0.77–1.40, p = 0.814).Microvascular obstruction is common in CA and is related to markers of amyloid infiltration. MVO is associated with an increased risk of mortality in AL‐CA, but not in ATTR‐CA. This reflects the intrinsic differences in disease biology between these two forms of CA, with MVO likely related to multiple myocardial processes, amyloid infiltration, oedema and myocyte death. [ABSTRACT FROM AUTHOR]

Published
2024
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21. Expansion of the National Amyloidosis Centre staging system to detect early mortality in transthyretin cardiac amyloidosis.

Author

Nitsche, Christian, Ioannou, Adam, Patel, Rishi K., Razvi, Yousuf, Porcari, Aldostefano, Rauf, Muhammad U., Bandera, Francesco, Aimo, Alberto, Emdin, Michele, Martinez‐Naharro, Ana, Venneri, Lucia, Petrie, Aviva, Wechalekar, Ashutosh, Lachmann, Helen, Hawkins, Philip N., Gillmore, Julian D., and Fontana, Marianna

Subjects
*RECEIVER operating characteristic curves, *CARDIAC amyloidosis, *GLOMERULAR filtration rate, *PEPTIDES, *HEART failure, *TRANSTHYRETIN, *DISEASE progression
Abstract

Aims: Transthyretin cardiac amyloidosis (ATTR‐CA) is stratified into prognostic categories using the National Amyloidosis Centre (NAC) staging system. The aims of this study were to further expand the existing NAC staging system to incorporate an additional disease stage that would identify patients at high risk of early mortality. Methods and results: The traditional NAC staging system (stage 1: N‐terminal pro‐B‐type natriuretic peptide [NT‐proBNP] ≤3000 ng/L and estimated glomerular filtration rate [eGFR] ≥45 ml/min; stage 3: NT‐proBNP >3000 ng/L and eGFR <45 ml/min; stage 2: remainder) was expanded by the introduction of a new stage 4 (defined as NT‐proBNP ≥10 000 ng/L irrespective of eGFR) and studied in 2042 patients. The optimal NT‐proBNP cut‐point was established using time‐dependent receiver operating characteristic curves in the subgroup of patients with NAC stage 3 disease. Mortality at 1 year according to NAC stage was 2.3% (n = 20/886) for stage 1, 8.8% (n = 62/706) for stage 2, 10.4% (n = 28/270) for stage 3, and 30.6% (n = 55/180) for stage 4 (log‐rank p < 0.001). After adjustment for age, mortality hazard for stage 4 was >15‐fold higher than that of stage 1 (hazard ratio [HR] 15.5; 95% confidence interval [CI] 9.3–26.1) and >3‐fold higher than that of stage 3 (HR 3.4; 95% CI 2.2–5.4). The increased risk of early mortality was consistent across the different genotypes and subclasses of patients based on the severity of heart failure symptoms and echocardiographic parameters. Conclusions: The proposed modification of the NAC staging system identifies patients with ATTR‐CA at a high risk of early mortality, who may benefit from a more intensive treatment strategy, and who are most likely to experience an event early in the course of a clinical trial. [ABSTRACT FROM AUTHOR]

Published
2024
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22. Cardiopulmonary Exercise Testing in Evaluating Transthyretin Amyloidosis

Author

Patel, Rishi K., primary, Bandera, Francesco, additional, Venneri, Lucia, additional, Porcari, Aldostefano, additional, Razvi, Yousuf, additional, Ioannou, Adam, additional, Chacko, Liza, additional, Martinez-Naharro, Ana, additional, Rauf, Muhammad U., additional, Knight, Daniel, additional, Brown, James, additional, Petrie, Aviva, additional, Wechalekar, Ashutosh, additional, Whelan, Carol, additional, Lachmann, Helen, additional, Muthurangu, Vivek, additional, Guazzi, Marco, additional, Hawkins, Philip N., additional, Gillmore, Julian D., additional, and Fontana, Marianna, additional

Published
2024
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23. Multiorgan Dysfunction and Associated Prognosis in Transthyretin Cardiac Amyloidosis

Author

Ioannou, Adam, primary, Nitsche, Christian, additional, Porcari, Aldostefano, additional, Patel, Rishi K., additional, Razvi, Yousuf, additional, Rauf, Muhammad U., additional, Martinez‐Naharro, Ana, additional, Venneri, Lucia, additional, Accietto, Antonella, additional, Netti, Lucrezia, additional, Bandera, Francesco, additional, Virsinskaite, Ruta, additional, Kotecha, Tushar, additional, Knight, Dan, additional, Petrie, Aviva, additional, Whelan, Carol, additional, Wechalekar, Ashutosh, additional, Lachmann, Helen, additional, Hawkins, Philip N., additional, Gillmore, Julian D., additional, and Fontana, Marianna, additional

Published
2024
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26. Myocardial perfusion in cardiac amyloidosis

Author

Chacko, Liza, primary, Kotecha, Tushar, additional, Ioannou, Adam, additional, Patel, Niket, additional, Martinez‐Naharro, Ana, additional, Razvi, Yousuf, additional, Patel, Rishi, additional, Massa, Paolo, additional, Venneri, Lucia, additional, Brown, James, additional, Porcari, Aldostefano, additional, Knott, Kristopher, additional, Manisty, Charlotte, additional, Knight, Daniel, additional, Lockie, Tim, additional, Rakhit, Roby, additional, Lachmann, Helen, additional, Wechelakar, Ashutosh, additional, Whelan, Carol, additional, Ponticos, Markella, additional, Moon, James, additional, González, Arantxa, additional, Gilbertson, Janet, additional, Riefolo, Mattia, additional, Leone, Ornella, additional, Xue, Hui, additional, Hawkins, Philip, additional, Kellman, Peter, additional, Gillmore, Julian, additional, and Fontana, Marianna, additional

Published
2024
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27. Cardiovascular Magnetic Resonance (CMR) in Transthyretin Amyloid Cardiomyopathy – a Study of Natural History of Treatment Response

Author

Patel, Rishi, primary, Martinez-Naharro, Ana, additional, Razvi, Yousuf, additional, Ioannou, Adam, additional, Porcari, Aldostefano, additional, Chacko, Liza, additional, Brown, James, additional, Knight, Daniel, additional, Kotecha, Tushar, additional, Manisty, Charlotte, additional, Moon, James, additional, Lachmann, Helen, additional, Wechalekar, Ashutosh, additional, Whelan, Carol, additional, Venneri, Lucia, additional, Kellman, Peter, additional, Hawkins, Philip, additional, Gillmore, Julian, additional, and Fontana, Marianna, additional

Published
2024
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28. Albuminuria in cardiac ATTR amyloidosis: prevalence, progression and prognostic importance

Author

Ioannou, Adam, primary, Rauf, Muhammad U, additional, Patel, Rishi K, additional, Razvi, Yousuf, additional, Porcari, Aldostefano, additional, Martinez‐Naharro, Ana, additional, Venneri, Lucia, additional, Bandera, Francesco, additional, Virsinskaite, Ruta, additional, Kotecha, Tushar, additional, Knight, Dan, additional, Petrie, Aviva, additional, Whelan, Carol, additional, Wechalekar, Ashutosh, additional, Lachmann, Helen, additional, Hawkins, Philip N, additional, Solomon, Scott D, additional, Gillmore, Julian D, additional, and Fontana, Marianna, additional

Published
2023
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29. Deep phenotyping of p.(V142I)‐associated variant ATTR amyloid cardiomyopathy: distinct from wild‐type ATTR amyloidosis?

Author

Razvi, Yousuf, primary, Ioannou, Adam, additional, Patel, Rishi K, additional, Chacko, Liza, additional, Karia, Nina, additional, Riefolo, Mattia, additional, Porcari, Aldostefano, additional, Rauf, Muhammad Umaid, additional, Starr, Neasa, additional, Ganesananthan, Sashiananthan, additional, Blakeney, Iona, additional, Kaza, Nandita, additional, Filisetti, Stefano, additional, Bolhuis, Roos Eline, additional, Rowczenio, Dorota, additional, Gilbertson, Janet, additional, Hutt, David, additional, Mahmood, Shameem, additional, Lachmann, Helen J, additional, Wechalekar, Ashutosh D, additional, Kotecha, Tushar, additional, Knight, Daniel S, additional, Coghlan, John G, additional, Petrie, Aviva, additional, Whelan, Carol J, additional, Venneri, Lucia, additional, Martinez‐Naharro, Ana, additional, Hawkins, Phillip, additional, Fontana, Marianna, additional, and Gillmore, Julian D, additional

Published
2023
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32. Impact of point-of-care pre-procedure creatinine and eGFR testing in patients with ST segment elevation myocardial infarction undergoing primary PCI: The pilot STATCREAT study

Author

Karamasis, Grigoris V., Hampton-Till, James, Al-Janabi, Firas, Mohdnazri, Shah, Parker, Michael, Ioannou, Adam, Jagathesan, Rohan, Kabir, Alagmir, Sayer, Jeremy W., Robinson, Nicholas M., Aggarwal, Rajesh K., Clesham, Gerald J., Gamma, Reto A., Kelly, Paul A., Tang, Kare H., Davies, John R., and Keeble, Thomas

Published
2017
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33. Deep phenotyping of p.(V142I)‐associated variant transthyretin amyloid cardiomyopathy: Distinct from wild‐type transthyretin amyloidosis?

Author

Razvi, Yousuf, Ioannou, Adam, Patel, Rishi K., Chacko, Liza, Karia, Nina, Riefolo, Mattia, Porcari, Aldostefano, Rauf, Muhammad Umaid, Starr, Neasa, Ganesananthan, Sashiananthan, Blakeney, Iona, Kaza, Nandita, Filisetti, Stefano, Bolhuis, Roos Eline, Rowczenio, Dorota, Gilbertson, Janet, Hutt, David, Mahmood, Shameem, Lachmann, Helen J., and Wechalekar, Ashutosh D.

Subjects
*CARDIAC amyloidosis, *TRANSTHYRETIN, *CARDIAC magnetic resonance imaging, *AMYLOID, *RIGHT ventricular dysfunction, *CARDIOMYOPATHIES
Abstract

Aims: Transthyretin amyloid cardiomyopathy (ATTR‐CM) is an increasingly recognized cause of heart failure. A total of 3–4% of individuals of African descent carry a TTR gene mutation encoding the p.(V142I) variant, a powerful risk factor for development of variant ATTR‐CM (ATTRv‐CM); this equates to 1.6 million carriers in the United States. We undertook deep phenotyping of p.(V142I)‐ATTRv‐CM and comparison with wild‐type ATTR‐CM (ATTRwt‐CM). Methods and results: A retrospective study of 413 patients with p.(V142I) ATTRv‐CM who attended the UK National Amyloidosis Centre (NAC) was conducted. Patients underwent evaluation at time of diagnosis, including clinical, echocardiography, and biomarker analysis; a subgroup had cardiac magnetic resonance (CMR) imaging. A total of 413 patients with ATTRwt‐CM, matched for independent predictors of prognosis (age, NAC Stage, decade of first presentation), were used as a comparator group. At time of diagnosis, patients with ATTRv‐CM had significant functional impairment by New York Heart Association classification (NHYA class ≥ III; 38%) and 6‐min walk test distance (median 276 m). Median 5‐year survival in ATTRv‐CM patients was 31 versus 59 months in matched patients with ATTRwt‐CM (p < 0.001). Patients with ATTRv‐CM had significant impairment of functional parameters by echocardiography including biventricular impairment, high burden of regurgitant valvular disease and low cardiac output. Multivariable analysis revealed the prognostic importance of right ventricular dysfunction. CMR and histological analysis revealed myocyte atrophy and widespread myocardial infiltration in ATTRv‐CM. Conclusion: p.(V142I)‐ATTRv‐CM has an aggressive phenotype characterized by myocyte loss and widespread myocardial infiltration which may account for frequent biventricular failure and poor prognosis in this ATTR‐CM genotypic subgroup. [ABSTRACT FROM AUTHOR]

Published
2024
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37. Tracking Treatment Response in Cardiac Light-Chain Amyloidosis With Native T1 Mapping

Author

Ioannou, Adam, primary, Patel, Rishi K., additional, Martinez-Naharro, Ana, additional, Razvi, Yousuf, additional, Porcari, Aldostefano, additional, Rauf, Muhammad U., additional, Bolhuis, Roos E., additional, Fernando-Sayers, Jacob, additional, Virsinskaite, Ruta, additional, Bandera, Francesco, additional, Kotecha, Tushar, additional, Venneri, Lucia, additional, Knight, Daniel, additional, Manisty, Charlotte, additional, Moon, James, additional, Lachmann, Helen, additional, Whelan, Carol, additional, Kellman, Peter, additional, Hawkins, Philip N., additional, Gillmore, Julian D., additional, Wechalekar, Ashutosh, additional, and Fontana, Marianna, additional

Published
2023
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38. Antibody-Associated Reversal of ATTR Amyloidosis–Related Cardiomyopathy

Author

Fontana, Marianna, primary, Gilbertson, Janet, additional, Verona, Guglielmo, additional, Riefolo, Mattia, additional, Slamova, Ivana, additional, Leone, Ornella, additional, Rowczenio, Dorota, additional, Botcher, Nicola, additional, Ioannou, Adam, additional, Patel, Rishi K., additional, Razvi, Yousuf, additional, Martinez-Naharro, Ana, additional, Whelan, Carol J., additional, Venneri, Lucia, additional, Duhlin, Amanda, additional, Canetti, Diana, additional, Ellmerich, Stephan, additional, Moon, James C., additional, Kellman, Peter, additional, Al-Shawi, Raya, additional, McCoy, Laura, additional, Simons, J. Paul, additional, Hawkins, Philip N., additional, and Gillmore, Julian D., additional

Published
2023
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39. Conventional heart failure therapy in cardiac ATTR amyloidosis

Author

Ioannou, Adam, primary, Massa, Paolo, additional, Patel, Rishi K, additional, Razvi, Yousuf, additional, Porcari, Aldostefano, additional, Rauf, Muhammad U, additional, Jiang, Anita, additional, Cabras, Giacomo, additional, Filisetti, Stefano, additional, Bolhuis, Roos E, additional, Bandera, Francesco, additional, Venneri, Lucia, additional, Martinez-Naharro, Ana, additional, Law, Steven, additional, Kotecha, Tushar, additional, Virsinskaite, Ruta, additional, Knight, Daniel S, additional, Emdin, Michele, additional, Petrie, Aviva, additional, Lachmann, Helen, additional, Wechelakar, Ashutosh, additional, Petrie, Mark, additional, Hughes, Alun, additional, Freemantle, Nick, additional, Hawkins, Philip N, additional, Whelan, Carol, additional, McMurray, John J V, additional, Gillmore, Julian D, additional, and Fontana, Marianna, additional

Published
2023
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40. Kiosk 1R-FB-04 - Cardiovascular Magnetic Resonance (CMR) in Transthyretin Amyloid Cardiomyopathy – a Study of Natural History of Treatment Response

Author

Patel, Rishi, Martinez-Naharro, Ana, Razvi, Yousuf, Ioannou, Adam, Porcari, Aldostefano, Chacko, Liza, Brown, James, Knight, Daniel, Kotecha, Tushar, Manisty, Charlotte, Moon, James, Lachmann, Helen, Wechalekar, Ashutosh, Whelan, Carol, Venneri, Lucia, Kellman, Peter, Hawkins, Philip, Gillmore, Julian, and Fontana, Marianna

Published
2024
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42. Full blood count as potential predictor of outcomes in patients undergoing cardiac resynchronization therapy

Author

Papageorgiou, Nikolaos, Falconer, Debbie, Ioannou, Adam, Wongwarawipat, Tanakal, Barra, Sergio, Tousoulis, Dimitris, Lim, Wei Yao, Khan, Fakhar Z., Ahsan, Syed, Muthumala, Amal, Hunter, Ross J., Finlay, Malcolm, Creta, Antonio, Rowland, Edward, Lowe, Martin, Segal, Oliver R., Schilling, Richard J., Lambiase, Pier D., Chow, Anthony W., and Providência, Rui

Published
2019
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43. Albuminuria in transthyretin cardiac amyloidosis: Prevalence, progression and prognostic importance.

Author

Ioannou, Adam, Rauf, Muhammad U., Patel, Rishi K., Razvi, Yousuf, Porcari, Aldostefano, Martinez‐Naharro, Ana, Venneri, Lucia, Bandera, Francesco, Virsinskaite, Ruta, Kotecha, Tushar, Knight, Dan, Petrie, Aviva, Whelan, Carol, Wechalekar, Ashutosh, Lachmann, Helen, Hawkins, Philip N., Solomon, Scott D., Gillmore, Julian D., and Fontana, Marianna

Subjects
*CARDIAC amyloidosis, *BRAIN natriuretic factor, *ALBUMINURIA, *TRANSTHYRETIN, *CHRONIC kidney failure, *GLOMERULAR filtration rate
Abstract

Aims: Transthyretin cardiac amyloidosis (ATTR‐CA) is an infiltrative cardiomyopathy that commonly presents with concomitant chronic kidney disease. Albuminuria is common in heart failure and associated with worse outcomes, but its prevalence and relationship to outcome in ATTR‐CA remains unclear. Methods and results: A total of 1181 patients with ATTR‐CA were studied (mean age 78.1 ± 7.9 years; 1022 [86.5%] male; median estimated glomerular filtration rate 59 ml/min/1.73m2 [interquartile range: 47–74]). Albuminuria was present in 563 (47.7%) patients (499 [88.6%] with microalbuminuria and 64 [11.4%] with macroalbuminuria). Patients with albuminuria had a more severe cardiac phenotype evidenced by higher serum cardiac biomarkers (median N‐terminal pro‐B‐type natriuretic peptide [NT‐proBNP]: 4027 ng/L [2173–6889] vs. 1851 ng/L [997–3209], p < 0.001; median troponin T: 69 ng/L [46–101] vs. 48 ng/L [34–68], p < 0.001) and worse echocardiographic indices of systolic (longitudinal strain: −10.0 ± 3.6% vs. −11.6 ± 3.8%, p < 0.001) and diastolic function (E/e′: 17.5 ± 6.4 vs. 16.4 ± 6.7, p < 0.001) than those with a normal urinary albumin to creatinine ratio (UACR). Microalbuminuria and macroalbuminuria were independently associated with mortality in the overall population (hazard ratio [HR] 1.47, 95% confidence interval [CI] 1.13–1.92, p = 0.005 and HR 1.87, 95% CI 1.15–3.05, p = 0.012, respectively). In a subgroup of patients (n = 349) without concomitant hypertension, diabetes mellitus or chronic kidney disease, albuminuria was also associated with mortality (HR 2.98, 95% CI 1.72–5.17, p < 0.001). At 12 months, 330 patients had a repeat UACR measurement; those in whom UACR increased by 30% or more (n = 148, 44.8%) had an increased risk of mortality (HR 1.84, 95% CI 1.06–3.19, p = 0.030). Conclusions: Albuminuria is common in patients with ATTR‐CA, and more prevalent in those with a more severe cardiac phenotype. Albuminuria at diagnosis and a significant increase in UACR during follow‐up are associated with mortality. [ABSTRACT FROM AUTHOR]

Published
2024
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44. Evolution of Disease-modifying Therapy for Transthyretin Cardiac Amyloidosis.

Author

Ioannou, Adam

Subjects
*HETEROCYCLIC compounds, *GENE therapy, *NONSTEROIDAL anti-inflammatory agents, *CARDIAC amyloidosis, *PATIENT safety, *PHARMACY information services, *AMYLOID, *DRUG efficacy, *DISEASE progression, *CHEMICAL inhibitors
Abstract

Transthyretin cardiac amyloidosis (ATTR-CA) represents an inexorably progressive and fatal cardiomyopathy. Increased understanding of the underlying pathogenesis responsible for the misfolding of transthyretin and the subsequent accumulation of amyloid fibrils within the myocardium has led to the development of several disease-modifying therapies that act on different stages of the disease pathway. Tafamidis is the first, and to date remains the only, therapy approved for the treatment of ATTR-CA, which, alongside acoramidis, stabilizes the transthyretin tetramer, preventing disaggregation, misfolding and formation of amyloid fibrils. Gene-silencing agents, such as patisiran, vutrisian and eplontersen, and novel gene-editing therapies, such as NTLA-2001, act to reduce the hepatic synthesis of transthyretin. Anti-amyloid therapies represent another strategy in the treatment of ATTR-CA and are designed to bind amyloid fibril epitopes and stimulate macrophage-mediated removal of amyloid fibrils from the myocardium. Many of these treatments are at an early investigational stage but represent an important area of unmet clinical need and could potentially reverse disease and restore cardiac functions even in patients with advanced disease. [ABSTRACT FROM AUTHOR]

Published
2024
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46. Tc-99m labelled bone scintigraphy in suspected cardiac amyloidosis

Author

Rauf, Muhammad Umaid, primary, Hawkins, Philip N, additional, Cappelli, Francesco, additional, Perfetto, Federico, additional, Zampieri, Mattia, additional, Argiro, Alessia, additional, Petrie, Aviva, additional, Law, Steven, additional, Porcari, Aldostefano, additional, Razvi, Yousuf, additional, Bomsztyk, Joshua, additional, Ravichandran, Sriram, additional, Ioannou, Adam, additional, Patel, Rishi, additional, Starr, Neasa, additional, Hutt, David F, additional, Mahmood, Shameem, additional, Wisniowski, Brendan, additional, Martinez–Naharro, Ana, additional, Venneri, Lucia, additional, Whelan, Carol, additional, Roczenio, Dorota, additional, Gilbertson, Janet, additional, Lachmann, Helen J, additional, Wechalekar, Ashutosh D, additional, Rapezzi, Claudio, additional, Serenelli, Matteo, additional, Massa, Paolo, additional, Caponetti, Angelo Giuseppe, additional, Ponziani, Alberto, additional, Accietto, Antonella, additional, Giovannetti, Alessandro, additional, Saturi, Giulia, additional, Sguazzotti, Maurizio, additional, Gagliardi, Christian, additional, Biagini, Elena, additional, Longhi, Simone, additional, Fontana, Marianna, additional, and Gillmore, Julian D, additional

Published
2023
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47. Prevalence, characteristics and outcomes of older patients with hereditary versus wild‐type transthyretin amyloid cardiomyopathy

Author

Porcari, Aldostefano, primary, Razvi, Yousuf, additional, Masi, Ambra, additional, Patel, Rishi, additional, Ioannou, Adam, additional, Rauf, Muhammad U., additional, Hutt, David F., additional, Rowczenio, Dorota, additional, Gilbertson, Janet, additional, Martinez‐Naharro, Ana, additional, Venneri, Lucia, additional, Whelan, Carol, additional, Lachmann, Helen, additional, Wechalekar, Ashutosh, additional, Quarta, Candida Cristina, additional, Merlo, Marco, additional, Sinagra, Gianfranco, additional, Hawkins, Philip N., additional, Fontana, Marianna, additional, and Gillmore, Julian D., additional

Published
2023
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48. Cardiac transplantation in transthyretin amyloid cardiomyopathy: Outcomes from three decades of tertiary center experience

Author

Razvi, Yousuf, primary, Porcari, Aldostefano, additional, Di Nora, Concetta, additional, Patel, Rishi K., additional, Ioannou, Adam, additional, Rauf, Muhammad U., additional, Masi, Ambra, additional, Law, Steven, additional, Chacko, Liza, additional, Rezk, Tamer, additional, Ravichandran, Sriram, additional, Gilbertson, Janet, additional, Rowczenio, Dorota, additional, Blakeney, Iona J., additional, Kaza, Nandita, additional, Hutt, David F., additional, Lachmann, Helen, additional, Wechalekar, Ashutosh, additional, Moody, William, additional, Lim, Sern, additional, Chue, Colin, additional, Whelan, Carol, additional, Venneri, Lucia, additional, Martinez-Naharro, Ana, additional, Merlo, Marco, additional, Sinagra, Gianfranco, additional, Livi, Ugolino, additional, Hawkins, Philip, additional, Fontana, Marianna, additional, and Gillmore, Julian D., additional

Published
2023
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49. 189 PREVALENCE, DISEASE CHARACTERISTICS AND OUTCOMES OF HEREDITARY TRANSTHYRETIN AMYLOID CARDIOMYOPATHY AMONG ELDERLY INDIVIDUALS

Author

Porcari, Aldostefano, primary, Razvi, Yousuf, additional, Masi, Ambra, additional, Patel, Rishi, additional, Ioannou, Adam, additional, Rauf, Muhammad Umaid, additional, Hutt, David F, additional, Rowczenio, Dorota, additional, Gilbertson, Janet, additional, Martinez-naharro, Ana, additional, Venneri, Lucia, additional, Whelan, Carol, additional, Lachmann, Helen, additional, Wechalekar, Ashutosh, additional, Quarta, Candida Cristina, additional, Merlo, Marco, additional, Sinagra, Gianfranco, additional, Hawkins, Philip N, additional, Fontana, Marianna, additional, and Gillmore, Julian D, additional

Published
2022
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50. Impact of Earlier Diagnosis in Cardiac ATTR Amyloidosis Over the Course of 20 Years

Author

Ioannou, Adam, primary, Patel, Rishi K., additional, Razvi, Yousuf, additional, Porcari, Aldostefano, additional, Sinagra, Gianfranco, additional, Venneri, Lucia, additional, Bandera, Francesco, additional, Masi, Ambra, additional, Williams, Georgina E., additional, O’Beara, Sophie, additional, Ganesananthan, Sharmananthan, additional, Massa, Paolo, additional, Knight, Daniel, additional, Martinez-Naharro, Ana, additional, Kotecha, Tushar, additional, Chacko, Liza, additional, Brown, James, additional, Rauf, Muhammad U., additional, Manisty, Charlotte, additional, Moon, James, additional, Lachmann, Helen, additional, Wechelakar, Ashutosh, additional, Petrie, Aviva, additional, Whelan, Carol, additional, Hawkins, Philip N., additional, Gillmore, Julian D., additional, and Fontana, Marianna, additional

Published
2022
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