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  • 1
    Article
    Article
    BMJ Publishing Group Ltd and British Cardiovascular Society
    Language: English
    In: Heart, 11 November 2012, Vol.98(21), p.1546
    Description: Cardiac amyloidosis of transthyretin fibril protein (ATTR) type is an infiltrative cardiomyopathy characterised by ventricular wall thickening and diastolic heart failure. Increased access to cardiovascular magnetic resonance imaging has led to a marked increase in referrals to our centre of Caucasian patients with wild-type ATTR (senile systemic) amyloidosis and Afro-Caribbean patients with the hereditary ATTR V122I type. Both subtypes present predominantly as isolated cardiomyopathy. The differential diagnosis includes cardiac amyloid light-chain (AL) amyloidosis, which has a poorer prognosis and can be amenable to chemotherapy. We review here the clinical features of cardiac ATTR amyloidosis and describe the diagnostic tests to determine ATTR type. Correct diagnosis is ever more crucial given that several novel therapies for ATTR amyloidosis are on the near horizon.
    Keywords: Cardiac Amyloidosis ; Cardiovascular Mri ; Cardiomyopathy ; Valvular Disease ; Cardiac Function ; Cardiac Remodelling ; Interventional Cardiology ; Non-Coronary Intervention ; Percutaneous Valve Therapy ; Heart Failure ; Systolic Heart Failure ; Diastolic Dysfunction ; Myocardial Disease ; Cardiomyopathy Restrictive
    ISSN: 1355-6037
    ISSN: 13556037
    E-ISSN: 1468-201X
    E-ISSN: 1468201X
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  • 2
    Language: English
    In: Circulation, Nov 25, 2014, Vol.130(22)
    Keywords: Electrocardiography – Usage ; Myocardial Diseases – Risk Factors ; Myocardial Diseases – Care and Treatment ; Heart Failure – Risk Factors ; Heart Failure – Care and Treatment ; Elderly – Health Aspects
    ISSN: 0009-7322
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  • 3
    In: Circulation, 2015, Vol.132(16), pp.1570-1579
    Description: BACKGROUND—: The prognosis and treatment of the 2 main types of cardiac amyloidosis, immunoglobulin light chain (AL) and transthyretin (ATTR) amyloidosis, are substantially influenced by cardiac involvement. Cardiovascular magnetic resonance with late gadolinium enhancement (LGE) is a reference standard for the diagnosis of cardiac amyloidosis, but its potential for stratifying risk is unknown. METHODS AND RESULTS—: Two hundred fifty prospectively recruited subjects, 122 patients with ATTR amyloid, 9 asymptomatic mutation carriers, and 119 patients with AL amyloidosis, underwent LGE cardiovascular magnetic resonance. Subjects were followed up for a mean of 24±13 months. LGE was performed with phase-sensitive inversion recovery (PSIR) and without (magnitude only). These were compared with extracellular volume measured with T1 mapping. PSIR was superior to magnitude-only inversion recovery LGE because PSIR always nulled the tissue (blood or myocardium) with the longest T1 (least gadolinium). LGE was classified into 3 patterns: none, subendocardial, and transmural, which were associated with increasing amyloid burden as defined by extracellular volume (P〈0.0001), with transitions from none to subendocardial LGE at an extracellular volume of 0.40 to 0.43 (AL) and 0.39 to 0.40 (ATTR) and to transmural at 0.48 to 0.55 (AL) and 0.47 to 0.59 (ATTR). Sixty-seven patients (27%) died. Transmural LGE predicted death (hazard ratio, 5.4; 95% confidence interval, 2.1–13.7; P〈0.0001) and remained independent after adjustment for N-terminal pro-brain natriuretic peptide, ejection fraction, stroke volume index, E/E′, and left ventricular mass index (hazard ratio, 4.1; 95% confidence interval, 1.3–13.1; P〈0.05). CONCLUSIONS—: There is a continuum of cardiac involvement in systemic AL and ATTR amyloidosis. Transmural LGE is determined reliably by PSIR and represents advanced cardiac amyloidosis. The PSIR technique provides incremental information on outcome even after adjustment for known prognostic factors.
    Keywords: Medicine ; Anatomy & Physiology;
    ISSN: 0009-7322
    E-ISSN: 15244539
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  • 4
    Language: English
    In: Journal of the American College of Cardiology, 27 March 2012, Vol.59(13), pp.E1225-E1225
    Description: To link to full-text access for this article, visit this link: http://dx.doi.org/10.1016/S0735-1097(12)61225-X Byline: Jason Dungu, Carol J. Whelan, Simon DJ Gibbs, Jennifer H. Pinney, Sanjay M. Banypersad, Christopher P. Venner, Helen J. Lachmann, Ashutosh Wechalekar, Julian D. Gillmore, Philip N. Hawkins, Lisa J. Anderson Author Affiliation: UK National Amyloidosis Centre, London, United Kingdom, St GeorgE's University of London, London, United Kingdom Article Note: (footnote) ACC Moderated Poster Contributions McCormick Place South, Hall A Saturday, March 24, 2012, 11:00 a.m.-Noon Session Title: Imaging: Established and Emerging Applications of MRI in Cardiomyopathies Abstract Category: 21. Imaging: MRI Presentation Number: 1087-79
    Keywords: Medicine
    ISSN: 0735-1097
    E-ISSN: 1558-3597
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  • 5
    Language: English
    In: Circulation, Nov 25, Vol.130(22)
    Keywords: Magnetic Resonance Imaging – Usage ; Amyloidosis – Risk Factors ; Amyloidosis – Diagnosis
    ISSN: 0009-7322
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  • 6
    Language: English
    In: Journal of the American College of Cardiology, 25 July 2017, Vol.70(4), pp.466-477
    Description: Cardiac transthyretin amyloidosis (ATTR) is an increasingly recognized cause of heart failure. Cardiac magnetic resonance (CMR), with late gadolinium enhancement (LGE) and T1 mapping, is emerging as a reference standard for diagnosis and characterization of cardiac amyloidosis. The authors used CMR with extracellular volume fraction (ECV) measurement to characterize cardiac involvement in relation to outcome in ATTR. Subjects comprised 263 patients with cardiac ATTR corroborated by grade 2 to 3 Tc-DPD ( Tc-3,3-diphosphono-1,2-propanodicarboxylic acid) cardiac uptake, 17 with suspected cardiac ATTR (grade 1 Tc-DPD), and 12 asymptomatic individuals with amyloidogenic transthyretin (TTR) mutations. Fifty patients with cardiac light-chain (AL) amyloidosis acted as disease comparators. Unlike cardiac AL amyloidosis, asymmetrical septal left ventricular hypertrophy (LVH) was present in 79% of patients with ATTR (70% sigmoid septum and 30% reverse septal contour), whereas symmetrical LVH was present in 18%, and 3% had no LVH. In patients with cardiac amyloidosis, the pattern of LGE was always typical for amyloidosis (29% subendocardial, 71% transmural), including right ventricular LGE (96%). During follow-up (19 ± 14 months), 65 patients died. ECV independently correlated with mortality and remained independent after adjustment for age, N-terminal pro-B-type natriuretic peptide, ejection fraction, E/E′, and left ventricular mass (hazard ratio: 1.164; 95% confidence interval: 1.066 to 1.271; p 〈 0.01). Asymmetrical hypertrophy, traditionally associated with hypertrophic cardiomyopathy, was the commonest pattern of ventricular remodeling in ATTR. LGE imaging was typical in all patients with cardiac ATTR. ECV correlated with amyloid burden and was an independent prognostic factor for survival in this cohort of patients.
    Keywords: Attr ; Extracellular Volume Fraction ; Late Gadolinium Enhancement ; Left Ventricular Hypertrophy ; N-Terminal Pro-B-Type Natriuretic Peptide ; Medicine
    ISSN: 0735-1097
    E-ISSN: 1558-3597
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  • 7
    Language: English
    In: American Heart Journal, July 2012, Vol.164(1), pp.72-79
    Description: About 4% of African Americans possess the isoleucine 122 (V122I) variant of transthyretin, associated with cardiac amyloidosis beyond ages of 55 to 60 years. Transthyretin amyloidosis associated with variant V122I (ATTR V122I) is likely to be an important cause of heart failure in Afro-Caribbean populations, but the high prevalence of left ventricular hypertrophy (LVH) and lack of awareness of this genetic disorder pose diagnostic hurdles. We report the electrocardiographic (ECG) features of ATTR V122I in the largest clinical series to date. Patients with ATTR V122I were identified in collaboration with the UK National Amyloidosis Centre. The ECG at presentation was assessed for cardiac rhythm, axis, and voltage complex size. We include 64 patients with ATTR V122I, with a median age of 74 years (range, 57-88 years). Normal or increased ECG voltage was present in 44.3% of patients, and overall 25% met the criteria for LVH. A significant negative correlation between voltage complex size and duration of illness was seen ( 〈 .05). First-degree heart block was evident in 56% of patients in sinus rhythm. During follow-up (n = 17; median, 28 months), 50% of patients with initial first-degree heart block required pacing. Electrocardiographic voltages meet the criteria for LVH in one quarter of patients with ATTR V122I cardiac amyloidosis. The widely held belief that cardiac amyloidosis is associated with low-voltage complexes is likely to contribute to underdiagnosis of ATTR V122I. First-degree heart block is common at diagnosis and identifies patients at high risk for subsequent pacing requirement.
    Keywords: Medicine
    ISSN: 0002-8703
    E-ISSN: 1097-6744
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  • 8
    Language: English
    In: Kidney International, December 2017, Vol.92(6), pp.1476-1483
    Description: Renal involvement causing progressive chronic kidney disease (CKD) is present in 70% of patients with systemic Ig light-chain (AL) amyloidosis at diagnosis. Chemotherapy that substantially suppresses free light chain production is associated with improved patient survival, but its benefit in delaying the onset of renal replacement therapy among patients who present with established advanced CKD has not been studied. To evaluate this, we studied 1000 patients enrolled in the prospective UK AL amyloidosis chemotherapy study (ALchemy). Of these, 84 patients had advanced amyloid-related CKD defined by an estimated glomerular filtration rate (eGFR) under 20 ml/min/1.73 m . We determined outcomes among these 84 patients, who had a median eGFR of 10 ml/min/1.73 m , in relation to response to chemotherapy evaluated at three, six, and 12 months from baseline. Patients who achieved suppression of 90% or more in their amyloidogenic free light chain (dFLC) within three months of baseline had significantly better overall survival, prolonged time to dialysis, and prolonged time to the composite endpoint of ‘death or dialysis’ compared to those who achieved lesser degrees of clonal response at the same time point. Even when this target of greater than 90% dFLC response was achieved but was delayed beyond 3 months, it was associated with worse outcomes. Cox regression analyses confirmed that a 90% or better dFLC response within 3 months was the only significant independent predictor of all three of these outcome measures. Thus, renal survival among patients with systemic immunologic light chain amyloidosis who present with advanced CKD is strongly dependent upon the magnitude and speed with which the underlying hematologic disorder is suppressed by chemotherapy.
    Keywords: Amyloid ; Amyloidosis ; Chemotherapy ; Chronic Kidney Disease ; Medicine
    ISSN: 0085-2538
    E-ISSN: 1523-1755
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  • 9
    In: Circulation, 2016, Vol.133(12), pp.e450-e451
    Description: We thank Aquaro, Cohen, and colleagues for their interest in our article.1 Cardiac involvement is a chief driver of prognosis in systemic amyloidosis, and the stratification of patients is essential for prognosis and choosing management strategies. Cardiovascular magnetic resonance with late gadolinium enhancement has good diagnostic accuracy for cardiac amyloidosis, but its prognostic impact was uncertain.2–6 This study confirms incremental prognostic information after adjusting for known prognosis factors. We note the comments of Aquaro and colleagues regarding cardiac biopsy, but point out that microscopic histological analyses of these tiny samples is not only open to sampling error, but, crucially, the presence of amyloid in heart muscle is not actually proof of cardiac amyloidosis. It is essential to make the distinction between the presence of amyloid deposits and the clinical syndromes of amyloidosis. Amyloid deposits occur widely throughout the tissues in patients with systemic amyloidosis, often without any clinical consequences, providing the basis for rectal, salivary gland, skin, and fat biopsies to support diagnosis. It is likely …
    Keywords: Medicine ; Anatomy & Physiology;
    ISSN: 0009-7322
    E-ISSN: 15244539
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  • 10
    Language: English
    In: Circulation, Nov 8, 2016, Vol.134(19)
    Keywords: Magnetic Resonance Imaging -- Usage ; Amyloidosis -- Care And Treatment ; Echocardiography -- Usage
    ISSN: 0009-7322
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