26 results on '"Wechelakar A"'
Search Results
2. Multi-Imaging Characterization of Cardiac Phenotype in Different Types of Amyloidosis
- Author
-
Ioannou, Adam, Patel, Rishi K., Razvi, Yousuf, Porcari, Aldostefano, Knight, Daniel, Martinez-Naharro, Ana, Kotecha, Tushar, Venneri, Lucia, Chacko, Liza, Brown, James, Manisty, Charlotte, Moon, James, Wisniowski, Brendan, Lachmann, Helen, Wechelakar, Ashutosh, Whelan, Carol, Kellman, Peter, Hawkins, Philip N., Gillmore, Julian D., and Fontana, Marianna
- Published
- 2023
- Full Text
- View/download PDF
3. Rare Forms of Cardiac Amyloidosis: Diagnostic Clues and Phenotype in Apo AI and AIV Amyloidosis
- Author
-
Ioannou, Adam, Porcari, Aldostefano, Patel, Rishi K., Razvi, Yousuf, Sinigiani, Giulio, Martinez-Naharro, Ana, Venneri, Lucia, Moon, James, Rauf, Muhammad U., Lachmann, Helen, Wechelakar, Ashutosh, Hawkins, Philip N., Gillmore, Julian D., and Fontana, Marianna
- Published
- 2023
- Full Text
- View/download PDF
4. Extracellular Volume Fraction by Computed Tomography Predicts Long-Term Prognosis Among Patients With Cardiac Amyloidosis
- Author
-
Gama, Francisco, Rosmini, Stefania, Bandula, Steve, Patel, Kush P., Massa, Paolo, Tobon-Gomez, Catalina, Ecke, Karolin, Stroud, Tyler, Condron, Mark, Thornton, George D., Bennett, Jonathan B., Wechelakar, Ashutosh, Gillmore, Julian D., Whelan, Carol, Lachmann, Helen, Taylor, Stuart A., Pugliese, Francesca, Fontana, Marianna, Moon, James C., Hawkins, Philip N., and Treibel, Thomas A.
- Published
- 2022
- Full Text
- View/download PDF
5. Clinical Importance of Left Atrial Infiltration in Cardiac Transthyretin Amyloidosis
- Author
-
Bandera, Francesco, Martone, Raffaele, Chacko, Liza, Ganesananthan, Sharmananthan, Gilbertson, Janet A., Ponticos, Markella, Lane, Thirusha, Martinez-Naharro, Ana, Whelan, Carol, Quarta, Cristina, Rowczenio, Dorota, Patel, Rishi, Razvi, Yousuf, Lachmann, Helen, Wechelakar, Ashutosh, Brown, James, Knight, Daniel, Moon, James, Petrie, Aviva, Cappelli, Francesco, Guazzi, Marco, Potena, Luciano, Rapezzi, Claudio, Leone, Ornella, Hawkins, Philip N., Gillmore, Julian D., and Fontana, Marianna
- Published
- 2022
- Full Text
- View/download PDF
6. 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
- Full Text
- View/download PDF
7. The clinical significance of transthyretin gene variant homozygosity in variant ATTR-CM
- Author
-
Razvi, Y, primary, Porcari, A, additional, Patel, R, additional, Ioannou, A, additional, Starr, N, additional, Rauf, M U, additional, Rowczenio, D, additional, Venneri, L, additional, Martinez-Naharro, A, additional, Whelan, C, additional, Lachmann, H, additional, Wechelakar, A, additional, Hawkins, P, additional, Fontana, M, additional, and Gillmore, J D, additional
- Published
- 2023
- Full Text
- View/download PDF
8. Multi-Imaging Characterization of Cardiac Phenotype in Different Types of Amyloidosis
- Author
-
Adam Ioannou, Rishi K. Patel, Yousuf Razvi, Aldostefano Porcari, Daniel Knight, Ana Martinez-Naharro, Tushar Kotecha, Lucia Venneri, Liza Chacko, James Brown, Charlotte Manisty, James Moon, Brendan Wisniowski, Helen Lachmann, Ashutosh Wechelakar, Carol Whelan, Peter Kellman, Philip N. Hawkins, Julian D. Gillmore, and Marianna Fontana
- Subjects
Radiology, Nuclear Medicine and imaging ,Cardiology and Cardiovascular Medicine - Published
- 2023
- Full Text
- View/download PDF
9. Extracellular Volume Fraction by Computed Tomography Predicts Long-Term Prognosis Among Patients With Cardiac Amyloidosis
- Author
-
Francisco Gama, Stefania Rosmini, Steve Bandula, Kush P. Patel, Paolo Massa, Catalina Tobon-Gomez, Karolin Ecke, Tyler Stroud, Mark Condron, George D. Thornton, Jonathan B. Bennett, Ashutosh Wechelakar, Julian D. Gillmore, Carol Whelan, Helen Lachmann, Stuart A. Taylor, Francesca Pugliese, Marianna Fontana, James C. Moon, Philip N. Hawkins, and Thomas A. Treibel
- Subjects
Radiology, Nuclear Medicine and imaging ,Cardiology and Cardiovascular Medicine - Abstract
Light chain (AL) and transthyretin (ATTR) amyloid fibrils are deposited in the extracellular space of the myocardium, resulting in heart failure and premature mortality. Extracellular expansion can be quantified by computed tomography, offering a rapid, cheaper, and more practical alternative to cardiac magnetic resonance, especially among patients with cardiac devices or on renal dialysis.This study sought to investigate the association of extracellular volume fraction by computed tomography (ECVPatients with confirmed systemic amyloidosis and varying degrees of cardiac involvement underwent electrocardiography-gated cardiac computed tomography. Whole heart and septal ECVA total of 72 patients were studied (AL: n = 35, ATTR: n = 37; median age: 67 [IQR: 59-76] years, 70.8% male). Mean septal ECVCardiac amyloid burden quantified by ECV
- Published
- 2022
- Full Text
- View/download PDF
10. Abstract 13148: Clinical Importance of Left Atrial Infiltration in Cardiac Transthyretin Amyloidosis
- Author
-
Bandera, Francesco, Martone, Raffaele, Chacko, Liza, Ganesananthan, Sharmananthan, Lane, Thirusha, Martinez-Naharro, Ana, Wheland, Carol, Quarta, Cristina, Rowczenio, Dorota, Ponticos, Markella, Gilbertson, Janet A, Lachmann, Helen, Wechelakar, Ashutosh, Brown, James, Knight, Daniel, James, Moon, Petrie, Aviva, Cappelli, Francesco, Guazzi, Marco, Potena, Luciano, Leone, Ornella, Hawkins, Philip N, Gillmore, Julian D, and Fontana, Marianna
- Published
- 2020
- Full Text
- View/download PDF
11. 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
- Full Text
- View/download PDF
12. Conventional heart failure therapy in cardiac ATTR amyloidosis
- Author
-
Adam Ioannou, Paolo Massa, Rishi K Patel, Yousuf Razvi, Aldostefano Porcari, Muhammad U Rauf, Anita Jiang, Giacomo Cabras, Stefano Filisetti, Roos E Bolhuis, Francesco Bandera, Lucia Venneri, Ana Martinez-Naharro, Steven Law, Tushar Kotecha, Ruta Virsinskaite, Daniel S Knight, Michele Emdin, Aviva Petrie, Helen Lachmann, Ashutosh Wechelakar, Mark Petrie, Alun Hughes, Nick Freemantle, Philip N Hawkins, Carol Whelan, John JV McMurray, Julian D Gillmore, and Marianna Fontana
- Subjects
Cardiology and Cardiovascular Medicine - Abstract
Background and Aims The aims of this study were to assess prescription patterns, dosages, discontinuation rates and association with prognosis of conventional heart failure (HF) medications in patients with transthyretin cardiac amyloidosis (ATTR-CA). Methods A retrospective analysis of all consecutive patients diagnosed with ATTR-CA at the National Amyloidosis Centre between 2000-2022 identified 2371 patients with ATTR-CA. Results Prescription of HF medications was greater among patients with a more severe cardiac phenotype, comprising beta-blockers in 55.4%, angiotensin-converting enzyme inhibitors (ACEi)/angiotensin-II receptor blockers (ARB) in 57.4%, and mineralocorticoid receptor antagonists (MRAs) in 39.0% of cases. During a median follow-up of 27.8 months (IQR 10.6-51.3), 21.7% had beta-blockers discontinued, and 32.9% had ACEi/ARB discontinued. In contrast, only 7.5% had MRAs discontinued. Propensity score-matched analysis demonstrated that treatment with MRAs was independently associated with a reduced risk of mortality in the overall population (HR 0.77 [95% CI 0.66-0.89], P40% (HR 0.75 [95% CI 0.63-0.90], P=0.002); and treatment with low-dose beta-blockers was independently associated with a reduced risk of mortality in a pre-specified subgroup of patients with a LVEF ≤40% (HR 0.61 [95% CI 0.45-0.83], P=0.002). No convincing differences were found for treatment with ACEi/ARBs. Conclusions Conventional HF medications are currently not widely prescribed in ATTR-CA, and those that received medication had more severe cardiac disease. Beta-blockers and ACEi/ARBs were often discontinued, but low-dose beta-blockers were associated with reduced risk of mortality in patients with a LVEF ≤40%. In contrast, MRAs were rarely discontinued and were associated with reduced risk of mortality in the overall population; but these findings require confirmation in prospective randomized controlled trials.
- Published
- 2023
- Full Text
- View/download PDF
13. Tracking Multiorgan Treatment Response in Systemic AL-Amyloidosis With Cardiac Magnetic Resonance Derived Extracellular Volume Mapping
- Author
-
Adam Ioannou, Rishi K. Patel, Ana Martinez-Naharro, Yousuf Razvi, Aldostefano Porcari, David F. Hutt, Francesco Bandera, Tushar Kotecha, Lucia Venneri, Liza Chacko, Paolo Massa, Melissa Hanger, Daniel Knight, Charlotte Manisty, James Moon, Cristina Quarta, Helen Lachmann, Carol Whelan, Peter Kellman, Philip N. Hawkins, Julian D. Gillmore, Ashutosh Wechelakar, and Marianna Fontana
- Subjects
Radiology, Nuclear Medicine and imaging ,Cardiology and Cardiovascular Medicine - Published
- 2023
- Full Text
- View/download PDF
14. Impact of Earlier Diagnosis in Cardiac ATTR Amyloidosis Over the Course of 20 Years
- Author
-
Adam Ioannou, Rishi K. Patel, Yousuf Razvi, Aldostefano Porcari, Gianfranco Sinagra, Lucia Venneri, Francesco Bandera, Ambra Masi, Georgina E. Williams, Sophie O’Beara, Sharmananthan Ganesananthan, Paolo Massa, Daniel Knight, Ana Martinez-Naharro, Tushar Kotecha, Liza Chacko, James Brown, Muhammad U. Rauf, Charlotte Manisty, James Moon, Helen Lachmann, Ashutosh Wechelakar, Aviva Petrie, Carol Whelan, Philip N. Hawkins, Julian D. Gillmore, Marianna Fontana, Ioannou, Adam, Patel, Rishi K, Razvi, Yousuf, Porcari, Aldostefano, Sinagra, Gianfranco, Venneri, Lucia, Bandera, Francesco, Masi, Ambra, Williams, Georgina E, O'Beara, Sophie, Ganesananthan, Sharmananthan, Massa, Paolo, Knight, Daniel, Martinez-Naharro, Ana, Kotecha, Tushar, Chacko, Liza, Brown, Jame, Rauf, Muhammad U, Manisty, Charlotte, Moon, Jame, Lachmann, Helen, Wechelakar, Ashutosh, Petrie, Aviva, Whelan, Carol, Hawkins, Philip N, Gillmore, Julian D, and Fontana, Marianna
- Subjects
amyloidosis ,amyloidosi ,Amyloid Neuropathies, Familial ,Settore MED/11 - Malattie dell'Apparato Cardiovascolare ,Stroke Volume ,transthyretin ,Ventricular Function, Left ,Cohort Studies ,Physiology (medical) ,Humans ,Prealbumin ,prognosis ,Cardiology and Cardiovascular Medicine ,Cardiomyopathies ,prognosi - Abstract
Background: Diagnostic and therapeutic advances have led to much greater awareness of transthyretin cardiac amyloidosis (ATTR-CA). We aimed to characterize changes in the clinical phenotype of patients diagnosed with ATTR-CA over the past 20 years. Methods: This is a retrospective observational cohort study of all patients referred to the National Amyloidosis Centre (2002–2021) in whom ATTR-CA was a differential diagnosis. Results: We identified 2995 patients referred with suspected ATTR-CA, of whom 1967 had a diagnosis of ATTR-CA confirmed. Analysis by 5-year periods revealed an incremental increase in referrals, with higher proportions of patients having been referred after bone scintigraphy and cardiac magnetic resonance imaging (2% versus 34% versus 51% versus 55%, chi-square P P P P P =0.01) and higher left ventricular ejection fraction (46.0%±8.9% versus 46.8%±11.0% versus 47.8%±11.0% versus 49.5%±11.1%, P P P P Conclusions: There has been a substantial increase in ATTR-CA diagnoses, with more patients being referred after local advanced cardiac imaging. Patients are now more often diagnosed at an earlier stage of the disease, with substantially lower mortality. These changes may have important implications for initiation and outcome of therapy and urgently need to be factored into clinical trial design.
- Published
- 2022
15. Progression of echocardiographic parameters and prognosis in transthyretin cardiac amyloidosis
- Author
-
Liza Chacko, Nina Karia, Lucia Venneri, Francesco Bandera, Beatrice Dal Passo, Lodovico Buonamici, Jonathan Lazari, Adam Ioannou, Aldostefano Porcari, Rishi Patel, Yousuf Razvi, James Brown, Daniel Knight, Ana Martinez‐Naharro, Carol Whelan, Candida C. Quarta, Charlotte Manisty, James Moon, Dorota Rowczenio, Janet A. Gilbertson, Helen Lachmann, Ashutosh Wechelakar, Aviva Petrie, William E. Moody, Richard P. Steeds, Luciano Potena, Mattia Riefolo, Ornella Leone, Claudio Rapezzi, Philip N. Hawkins, Julian D. Gillmore, Marianna Fontana, Chacko, Liza, Karia, Nina, Venneri, Lucia, Bandera, Francesco, Dal Passo, Beatrice, Buonamici, Lodovico, Lazari, Jonathan, Ioannou, Adam, Porcari, Aldostefano, Patel, Rishi, Razvi, Yousuf, Brown, Jame, Knight, Daniel, Martinez-Naharro, Ana, Whelan, Carol, Quarta, Candida C., Manisty, Charlotte, Moon, Jame, Rowczenio, Dorota, Gilbertson, Janet A., Lachmann, Helen, Wechelakar, Ashutosh, Petrie, Aviva, Moody, William E., Steeds, Richard P., Potena, Luciano, Riefolo, Mattia, Leone, Ornella, Rapezzi, Claudio, Hawkins, Philip N., Gillmore, Julian D., and Fontana, Marianna
- Subjects
Heart Failure ,Amyloid Neuropathies, Familial ,Cardiomyopathy ,Echocardiography ,Prognosis ,Progression ,Restrictive ,Prognosi ,Settore MED/11 - Malattie dell'Apparato Cardiovascolare ,Humans ,Prealbumin ,Cardiology and Cardiovascular Medicine ,Cardiomyopathies - Abstract
Aims: Transthyretin amyloid cardiomyopathy (ATTR-CM) is an increasingly diagnosed disease. Echocardiography is widely utilized, but studies to confirm the value of echocardiography for tracking changes over time are not available. We sought to describe (i) changes in multiple echocardiographic parameters; (ii) differences in rate of progression of three predominant genotypes; and (iii) the ability of changes in echocardiographic parameters to predict prognosis. Methods and results: We prospectively studied 877 ATTR-CM patients attending our centre between 2000 and 2020. Serial echocardiography findings at baseline, 12 months and 24 months were compared with survival. Overall, 565 patients had wild-type ATTR-CM and 312 hereditary ATTR-CM (201 with V122I; 90 with T60A). There was progressive worsening of structural and functional parameters over time, patients with V122I ATTR-CM showing more rapid worsening of left and right ventricular structural and functional parameters compared to both wild-type and T60A ATTR-CM. Among a wide range of echocardiographic analyses, including deformation-based parameters, only worsening in the degree of mitral (MR) and tricuspid regurgitation (TR) at 12- and 24-month assessments was associated with worse prognosis (change at 12 months: MR, hazard ratio 1.43 [95% confidence interval 1.14–1.80], p = 0.002; TR, hazard ratio 1.38 [95% confidence interval 1.10–1.75], p = 0.006). Worsening in MR remained independently associated with poor prognosis after adjusting for known predictors. Conclusion: In ATTR-CM, echocardiographic parameters progressively worsen over time. Patients with V122I ATTR-CM demonstrate the most rapid deterioration. Worsening of MR and TR were the only parameters associated with mortality, MR remaining independent after adjusting for known predictors.
- Published
- 2022
16. Extracellular volume fraction by computed tomography predicts long-term prognosis among patients with cardiac amyloidosis
- Author
-
F Gama, S Rosmini, S Bandula, K P Patel, G D Thornton, J B Bennett, A Wechelakar, J D Gillmore, C Whelan, H Lachmann, S Taylor, M Fontana, J Moon, P N Hawkins, and T Treibel
- Subjects
Cardiology and Cardiovascular Medicine - Abstract
Objective This study sought to investigate the association of extracellular volume fraction by computed tomography (ECVCT), myocardial remodeling and mortality in patients with systemic amyloidosis. Background Light chain (AL) and transthyretin (ATTR) amyloid fibrils are deposited in the extracellular space of the myocardium, resulting in heart failure and premature mortality. Extracellular expansion can be quantified by CT, offering a rapid and cost-effective alternative to cardiovascular magnetic resonance (CMR), especially among patients with cardiac devices or on renal dialysis. Methods Patients with confirmed systemic amyloidosis and varying degrees of cardiac involvement underwent ECG-gated cardiac CT. ECVCT was analysed in the inter-ventricular septum. All patients also underwent clinical assessment, ECG, echocardiography, serum amyloid protein component (SAP) and/or technetium-99m (99mTc) 3,3-diphosphono-1,2-propanodicarboxylic acid scintigraphy. ECVCT was compared across different extents of cardiac infiltration (ATTR Perugini Grade / AL Mayo Class) and evaluated for its association with myocardial remodelling and all-cause mortality. Results 72 patients were studied (AL n=35, ATTR n=37; age 67 (59–76) years, 71% males). Mean septal ECVCT was 42.7±13.1% and 55.8±10.9% in AL and ATTR, respectively, and correlated with indexed left ventricular (LV) mass (r=0.426, p Conclusion Cardiac amyloid burden quantified by ECVCT is associated with adverse cardiac remodelling as well as all-cause mortality among ATTR amyloid patients. ECVCT may address the need for better identification and risk stratification of amyloid patients, using a widely-accessible imaging modality (Figure 2). Funding Acknowledgement Type of funding sources: None.
- Published
- 2022
- Full Text
- View/download PDF
17. Prognostic implications of clinical phenotype and severity of cardiac involvement in patients presenting with immunoglobulin light chain amyloidosis
- Author
-
A Porcari, A Masi, A Ioannou, R K Patel, Y Razvi, L Venneri, A Martinez-Naharro, G Sinagra, A Wechelakar, P N Hawkins, J D Gillmore, and M Fontana
- Subjects
Cardiology and Cardiovascular Medicine - Abstract
Background Patients with systemic immunoglobulin light chain (AL) amyloidosis may present with a wide array of signs and symptoms due to the multi-systemic organ involved. The presence of cardiac involvement is the key determinant of survival. Cardiac magnetic resonance (CMR) has the unique ability to measure the continuum of cardiac amyloidosis (CA) infiltration providing a deep characterisation from early CA involvement to severe degree of CA burden. Purpose The aim of this study was to characterise the clinical profiles and the severity of organ involvement in patients presenting with AL amyloidosis and to investigate implications for long-term outcome. Methods Patients newly diagnosed with AL amyloidosis at the National Amyloidosis Centre underwent comprehensive clinical, laboratory and instrumental work up, including CMR imaging with left ventricular (LV) mass, late gadolinium enhancement (LGE) and extracellular volume (ECV). The clinical phenotypes were classified in cardiac, renal and other according to the symptoms at presentation. The degree of CA was investigated by CMR: 0= no features of CA (normal LV mass, no LGE and normal ECV); 1=early cardiac amyloid infiltration (normal LV mass, raised ECV no LGE); 2= characteristic of CA with normal mass (diffuse subendocardial or transmural LGE, altered gadolinium kinetics and raised ECV); 3= characteristic of CA with elevated mass (diffuse subendocardial or transmural LGE and raised ECV). The study outcome was all-cause mortality. Results The study population included 241 AL patients presenting with cardiac and renal (22.8%, n=55), cardiac (28.2%, n=68), renal (33.2%, n=80) and other (15.8% n=38) phenotypes. During a median follow up of 33 (IQR 7–52) months, cardiac phenotype either in isolation or in combination with renal phenotype was associated with a higher rate of all-cause mortality compared to the others (p Conclusions Patients with newly diagnosed AL amyloidosis present most frequently with renal and cardiac phenotypes. CMR detects CA in >40% of patients with non-cardiac phenotype. ECV is an independent predictor of all-cause mortality across the full clinical spectrum of AL amyloidosis. Funding Acknowledgement Type of funding sources: None.
- Published
- 2022
- Full Text
- View/download PDF
18. 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
- Full Text
- View/download PDF
19. Extracellular volume fraction by computed tomography predicts long-term prognosis among patients with cardiac amyloidosis
- Author
-
Gama, F, primary, Rosmini, S, additional, Bandula, S, additional, Patel, K P, additional, Thornton, G D, additional, Bennett, J B, additional, Wechelakar, A, additional, Gillmore, J D, additional, Whelan, C, additional, Lachmann, H, additional, Taylor, S, additional, Fontana, M, additional, Moon, J, additional, Hawkins, P N, additional, and Treibel, T, additional
- Published
- 2022
- Full Text
- View/download PDF
20. Prognostic implications of clinical phenotype and severity of cardiac involvement in patients presenting with immunoglobulin light chain amyloidosis
- Author
-
Porcari, A, primary, Masi, A, additional, Ioannou, A, additional, Patel, R K, additional, Razvi, Y, additional, Venneri, L, additional, Martinez-Naharro, A, additional, Sinagra, G, additional, Wechelakar, A, additional, Hawkins, P N, additional, Gillmore, J D, additional, and Fontana, M, additional
- Published
- 2022
- Full Text
- View/download PDF
21. Progression of echocardiographic parameters and prognosis in transthyretin cardiac amyloidosis
- Author
-
Chacko, Liza, primary, Karia, Nina, additional, Venneri, Lucia, additional, Bandera, Francesco, additional, Passo, Beatrice Dal, additional, Buonamici, Lodovico, additional, Lazari, Jonathan, additional, Ioannou, Adam, additional, Porcari, Aldostefano, additional, Patel, Rishi, additional, Razvi, Yousuf, additional, Brown, James, additional, Knight, Daniel, additional, Martinez‐Naharro, Ana, additional, Whelan, Carol, additional, Quarta, Candida C., additional, Manisty, Charlotte, additional, Moon, James, additional, Rowczenio, Dorota, additional, Gilbertson, Janet A., additional, Lachmann, Helen, additional, Wechelakar, Ashutosh, additional, Petrie, Aviva, additional, Moody, William E., additional, Steeds, Richard P., additional, Potena, Luciano, additional, Riefolo, Mattia, additional, Leone, Ornella, additional, Rapezzi, Claudio, additional, Hawkins, Philip N., additional, Gillmore, Julian D., additional, and Fontana, Marianna, additional
- Published
- 2022
- Full Text
- View/download PDF
22. Clinical Importance of Left Atrial Infiltration in Cardiac Transthyretin Amyloidosis
- Author
-
Luciano Potena, Dorota Rowczenio, Janet A. Gilbertson, Daniel R. Knight, James Brown, Raffaele Martone, Ornella Leone, Ashutosh Wechelakar, Claudio Rapezzi, Carol J. Whelan, James C. Moon, Aviva Petrie, Markella Ponticos, Thirusha Lane, Liza Chacko, Sharmananthan Ganesananthan, Julian D. Gillmore, Rishi K Patel, Ana Martinez-Naharro, Philip N. Hawkins, Helen J. Lachmann, Francesco Bandera, Francesco Cappelli, Cristina Quarta, Marco Guazzi, Yousuf Razvi, and Marianna Fontana
- Subjects
Pathology ,medicine.medical_specialty ,ROI, region of interest ,MCF, myocardial contraction factor ,ATTR-CM, transthyretin amyloid cardiomyopathy ,HF, heart failure ,LA, left atrium ,TAPSE, tricuspid annular plane systolic excursion ,NO ,MAPSE, mitral annular plane systolic excursion ,Muscular Diseases ,atrial function ,Predictive Value of Tests ,Left atrial ,atrial strain ,medicine ,Humans ,Prealbumin ,atrial histology ,echocardiography ,wtATTR, wild-type transthyretin ,Radiology, Nuclear Medicine and imaging ,Heart Atria ,Original Research ,amyloidosis ,Amyloid Neuropathies, Familial ,biology ,business.industry ,Amyloidosis ,atrial stiffness ,medicine.disease ,hATTR, hereditary ATTR amyloidosis ,PASP, pulmonary artery systolic pressure ,Transthyretin ,Atrial strain ,LV, left ventricle ,EMB, endomyocardial biopsy ,biology.protein ,LAS, left atrium strain ,Cardiology and Cardiovascular Medicine ,Amyloid cardiomyopathy ,business ,Infiltration (medical) - Abstract
Objectives The aim of this study was to characterize left atrial (LA) pathology in explanted hearts with transthyretin amyloid cardiomyopathy (ATTR-CM); LA mechanics using echocardiographic speckle-tracking in a large cohort of patients with ATTR-CM; and to study the association with mortality. Background The clinical significance of LA involvement in ATTR-CM is of great clinical interest. Methods Congo red staining and immunohistochemistry was performed to assess the presence, type, and extent of amyloid and associated changes in 5 explanted ATTR-CM atria. Echo speckle tracking was used to assess LA reservoir, conduit, contractile function, and stiffness in 906 patients with ATTR-CM (551 wild-type (wt)-ATTR-CM; 93 T60A-ATTR-CM; 241 V122I-ATTR-CM; 21 other). Results There was extensive ATTR amyloid infiltration in the 5 atria, with loss of normal architecture, vessels remodeling, capillary disruption, and subendocardial fibrosis. Echo speckle tracking in 906 patients with ATTR-CM demonstrated increased atrial stiffness (median [25th-75th quartile] 1.83 [1.15-2.92]) that remained independently associated with prognosis after adjusting for known predictors (lnLA stiff: HR: 1.23; 95% CI: 1.03-1.49; P = 0.029). There was substantial impairment of the 3 phasic functional atrial components (reservoir 8.86% [5.94%-12.97%]; conduit 6.5% [4.53%-9.28%]; contraction function 4.0% [2.29%-6.56%]). Atrial contraction was absent in 22.1% of patients whose electrocardiograms showed sinus rhythm (SR) “atrial electromechanical dissociation” (AEMD). AEMD was associated with poorer prognosis compared with patients with SR and effective mechanical contraction (P = 0.0018). AEMD conferred a similar prognosis to patients in atrial fibrillation. Conclusions The phenotype of ATTR-CM includes significant infiltration of the atrial walls, with progressive loss of atrial function and increased stiffness, which is a strong independent predictor of mortality. AEMD emerged as a distinctive phenotype identifying patients in SR with poor prognosis., Central Illustration
- Published
- 2022
23. The Authors’ Reply
- Author
-
Bandera, Francesco, primary, Martone, Raffaele, additional, Chacko, Liza, additional, Ganesananthan, Sharmananthan, additional, Gilbertson, Janet A., additional, Ponticos, Markella, additional, Lane, Thirusha, additional, Martinez-Naharro, Ana, additional, Whelan, Carol, additional, Quarta, Cristina, additional, Rowczenio, Dorota, additional, Patel, Rishi, additional, Razvi, Yousuf, additional, Lachmann, Helen, additional, Wechelakar, Ashutosh, additional, Brown, James, additional, Knight, Daniel, additional, Moon, James, additional, Petrie, Aviva, additional, Cappelli, Francesco, additional, Guazzi, Marco, additional, Potena, Luciano, additional, Rapezzi, Claudio, additional, Leone, Ornella, additional, Hawkins, Philip N., additional, Gillmore, Julian D., additional, and Fontana, Marianna, additional
- Published
- 2021
- Full Text
- View/download PDF
24. The Authors’ Reply
- Author
-
Francesco Bandera, Raffaele Martone, Liza Chacko, Sharmananthan Ganesananthan, Janet A. Gilbertson, Markella Ponticos, Thirusha Lane, Ana Martinez-Naharro, Carol Whelan, Cristina Quarta, Dorota Rowczenio, Rishi Patel, Yousuf Razvi, Helen Lachmann, Ashutosh Wechelakar, James Brown, Daniel Knight, James Moon, Aviva Petrie, Francesco Cappelli, Marco Guazzi, Luciano Potena, Claudio Rapezzi, Ornella Leone, Philip N. Hawkins, Julian D. Gillmore, and Marianna Fontana
- Subjects
Radiology, Nuclear Medicine and imaging ,Cardiology and Cardiovascular Medicine ,NO - Published
- 2021
- Full Text
- View/download PDF
25. Abstract 13148: Clinical Importance of Left Atrial Infiltration in Cardiac Transthyretin Amyloidosis
- Author
-
Philip N. Hawkins, Carol Wheland, Helen J. Lachmann, Markella Ponticos, Julian D. Gillmore, Dorota Rowczenio, Moon James, Francesco Bandera, Marianna Fontana, Francesco Cappelli, Cristina Quarta, Marco Guazzi, Ornella Leone, Ashutosh Wechelakar, Ana Martinez-Naharro, Thirusha Lane, James Brown, Luciano Potena, Janet A. Gilbertson, Raffaele Martone, Aviva Petrie, Liza Chacko, Sharmananthan Ganesananthan, and Daniel R. Knight
- Subjects
Pathology ,medicine.medical_specialty ,biology ,business.industry ,Amyloidosis ,Cardiomyopathy ,medicine.disease ,Transthyretin ,Left atrial ,Physiology (medical) ,medicine ,biology.protein ,Clinical significance ,Cardiology and Cardiovascular Medicine ,business ,Infiltration (medical) ,Attr amyloidosis ,Amyloid angiopathy - Abstract
Introduction: The clinical significance of left atrial (LA) involvement and dysfunction in ATTR amyloidosis cardiomyopathy (ATTR-CM) has not been characterized. We sought to study: 1) LA pathology in heart specimens from ATTR-CM patients 2) LA stiffness and mechanics using echocardiographic speckle tracking (EST) in cardiac ATTR-CM 3) the association between parameters of atrial function and mortality. Methods: Congo red staining and immunohistochemistry was performed to assess amyloid in the atria from 5 ATTR-CM heart specimens. 2D EST was used to assess LA reservoir, conduit, contractile function and stiffness in 906 ATTR-CM patients (551 wt ATTR; 93 T60A ATTR; 241 V122I ATTR; and 21 other gene variants). Results: There was extensive ATTR amyloid infiltration in the 5 atria, with loss of normal architecture, infiltration and remodelling of vessels, capillary disruption and subendocardial fibrosis. EST demonstrated increased atrial stiffness [median (25th-75th quartile) 1.83 (1.15-2.92)] that remained independently associated with reduced survival, after adjusting for known prognostic variables (lnLA stiff: HR= 1.26, CI 1.07-1.57; p=.009). There was substantial impairment of the three phasic functional atrial components [reservoir 8.86(5.94-12.97)%; conduit 6.5(4.53-9.28)%; and contraction 4.0(2.29-6.56)%]. Atrial contractile function was absent in 21.6% of patients whose ECG showed sinus rhythm (SR)-atrial electro-mechanical dissociation (AEMD). AEMD was associated with a poorer prognosis compared to SR patients who had effective mechanical contraction (p Conclusions: The phenotype of ATTR-CM includes clinically significant infiltration of the atrial walls characterized by progressive loss of function and increased stiffness, a strong independent predictor of mortality. AEMD emerged as a distinctive functional phenotype identifying patients in SR with poor prognosis.
- Published
- 2020
- Full Text
- View/download PDF
26. 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
- Full Text
- View/download PDF
Catalog
Discovery Service for Jio Institute Digital Library
For full access to our library's resources, please sign in.