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Article ; Online: Monitoring cardiac amyloidosis with multimodality imaging.

Starr, Neasa / Ioannou, Adam / Martinez-Naharro, Ana

Revista espanola de cardiologia (English ed.)

2023 Volume 77, Issue 1, Page(s) 79–87

Abstract: Cardiac amyloidosis (CA) refers to an infiltrative process involving amyloid fibril deposition in the myocardium causing restrictive cardiomyopathy. While various types can affect the heart, the predominant forms are immunoglobulin light-chain (AL) ... ...

Abstract	Cardiac amyloidosis (CA) refers to an infiltrative process involving amyloid fibril deposition in the myocardium causing restrictive cardiomyopathy. While various types can affect the heart, the predominant forms are immunoglobulin light-chain (AL) amyloidosis and transthyretin (ATTR) amyloidosis. This review article explores the expanding field of imaging techniques used to diagnose AL-CA and ATTR-CA, highlighting their usefulness in prognostication and disease surveillance. Echocardiography is often the initial imaging modality to suspect CA and, since the incorporation of nonbiopsy criteria using bone scintigraphy, diagnosing ATTR-CA has become more attainable following exclusion of plasma cell dyscrasia. Cardiac magnetic resonance is progressively emerging as a vital tool for imaging CA, and is used in diagnosis, prognostication, and disease surveillance. The use of cardiac magnetic resonance in AL-CA is discussed, as it has been shown to accurately evaluate organ response to chemotherapy. As novel drug treatments emerge in the realm of ATTR-CA, the use of cardiovascular imaging surveillance to monitor disease progression is discussed, as it is gaining prominence as a critical consideration. The ongoing phase III trials investigating treatments for patients with ATTR-CA, will undoubtedly enhance our understanding of cardiac imaging surveillance.
MeSH term(s)	Humans ; Amyloid Neuropathies, Familial/diagnosis ; Heart ; Myocardium/pathology ; Magnetic Resonance Imaging ; Amyloid ; Cardiomyopathies/metabolism
Chemical Substances	Amyloid
Language	Spanish
Publishing date	2023-09-09
Publishing country	Spain
Document type	Journal Article ; Review
ZDB-ID	2592481-3
ISSN	1885-5857 ; 1885-5857
ISSN (online)	1885-5857
ISSN	1885-5857
DOI	10.1016/j.rec.2023.06.014
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Article: Viral Myocarditis and Dilated Cardiomyopathy as a Consequence-Changing Insights from Advanced Imaging.

Kang, Nicolas / Friedrich, Matthias G / Abramov, Dmitry / Martinez-Naharro, Ana / Fontana, Marianna / Parwani, Purvi

Heart failure clinics

2023 Volume 19, Issue 4, Page(s) 445–459

Abstract: Advancements in quantitative cardiac magnetic resonance (CMR) have revolutionized the diagnosis and management of viral myocarditis. With the addition of T1 and T2 mapping parameters in the updated Lake Louise Criteria, CMR can diagnose myocarditis with ... ...

Abstract	Advancements in quantitative cardiac magnetic resonance (CMR) have revolutionized the diagnosis and management of viral myocarditis. With the addition of T1 and T2 mapping parameters in the updated Lake Louise Criteria, CMR can diagnose myocarditis with superior diagnostic accuracy compared with endomyocardial biopsy, especially in stable patients. Additionally, the unique value of CMR tissue characterization continues to improve the diagnosis and risk stratification of myocarditis. This review will discuss new and ongoing developments in cardiovascular imaging and its application to noninvasive diagnosis, prognostication, and management of viral myocarditis and its complications.
MeSH term(s)	Humans ; Cardiomyopathy, Dilated/diagnostic imaging ; Myocarditis/diagnostic imaging ; Heart ; Diagnostic Imaging ; Cardiac Catheterization
Language	English
Publishing date	2023-06-11
Publishing country	United States
Document type	Journal Article ; Review
ZDB-ID	2212019-1
ISSN	1551-7136
ISSN	1551-7136
DOI	10.1016/j.hfc.2023.03.009
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Article ; Online: Atrial Involvement in Cardiac Amyloidosis: Beyond Dilatation.

Fontana, Marianna / Patel, Rishi K / Martinez-Naharro, Ana

JACC. CardioOncology

2020 Volume 2, Issue 5, Page(s) 732–734

Language	English
Publishing date	2020-12-15
Publishing country	United States
Document type	Editorial ; Comment
ISSN	2666-0873
ISSN (online)	2666-0873
DOI	10.1016/j.jaccao.2020.11.005
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Article ; Online: Detailed Understating of Cardiac Amyloidosis by CMR: Towards Personalized Medicine.

Fontana, Marianna / Chacko, Liza A / Martinez-Naharro, Ana

JACC. Cardiovascular imaging

2020 Volume 13, Issue 6, Page(s) 1311–1313

MeSH term(s)	Amyloidosis/diagnostic imaging ; Cardiomyopathies/diagnostic imaging ; Contrast Media ; Gadolinium ; Humans ; Precision Medicine
Chemical Substances	Contrast Media ; Gadolinium (AU0V1LM3JT)
Language	English
Publishing date	2020-06-01
Publishing country	United States
Document type	Editorial ; Research Support, Non-U.S. Gov't ; Comment
ZDB-ID	2491503-8
ISSN	1876-7591 ; 1936-878X
ISSN (online)	1876-7591
ISSN	1936-878X
DOI	10.1016/j.jcmg.2020.04.013
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Article ; Online: Diagnostic imaging of cardiac amyloidosis.

Martinez-Naharro, Ana / Baksi, A John / Hawkins, Philip N / Fontana, Marianna

Nature reviews. Cardiology

2020 Volume 17, Issue 7, Page(s) 413–426

Abstract: Systemic amyloidosis encompasses a debilitating, under-diagnosed but increasingly recognized group of disorders characterized by the extracellular deposition of misfolded proteins in one or more organs. Cardiac amyloid deposition leads to an infiltrative ...

Abstract	Systemic amyloidosis encompasses a debilitating, under-diagnosed but increasingly recognized group of disorders characterized by the extracellular deposition of misfolded proteins in one or more organs. Cardiac amyloid deposition leads to an infiltrative or restrictive cardiomyopathy and is the major contributor to poor prognosis in patients with systemic amyloidosis. In total, >30 proteins can form amyloid fibrils, but the two main types of amyloid that can infiltrate the heart are monoclonal immunoglobulin light-chain amyloid and transthyretin amyloid. Cardiac amyloidosis can be acquired in older individuals or inherited from birth. Given the nonspecific symptoms of these disorders, a high index of suspicion is paramount in making the correct diagnosis, which can involve the use of non-invasive imaging methods such as echocardiography, bone scintigraphy and cardiovascular MRI. In the past decade, the use of cardiovascular MRI with tissue characterization and bone scintigraphy to diagnose cardiac amyloidosis has revolutionized our understanding of the disease, leading to changes in patient care. However, a need remains for improved awareness and expertise, and greater clinical suspicion, because the initial clues provided by electrocardiography and echocardiography might not be typical. With specific treatments now available, timely diagnosis of cardiac amyloidosis is more important than ever. In this Review, we discuss the current and novel approaches for the diagnostic imaging of cardiac amyloidosis.
MeSH term(s)	Amyloidosis/diagnostic imaging ; Echocardiography ; Heart Diseases/diagnostic imaging ; Humans ; Magnetic Resonance Imaging ; Radionuclide Imaging
Language	English
Publishing date	2020-02-10
Publishing country	England
Document type	Journal Article ; Research Support, Non-U.S. Gov't ; Review
ZDB-ID	2490375-9
ISSN	1759-5010 ; 1759-5002
ISSN (online)	1759-5010
ISSN	1759-5002
DOI	10.1038/s41569-020-0334-7
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Article ; Online: Cardiac amyloidosis.

Martinez-Naharro, Ana / Hawkins, Philip N / Fontana, Marianna

Clinical medicine (London, England)

2018 Volume 18, Issue Suppl 2, Page(s) s30–s35

Abstract: Systemic amyloidosis comprises an uncommon group of disorders caused by the extracellular deposition of misfolded proteins in various organs. Cardiac amyloid deposition, causing an infiltrative/restrictive cardiomyopathy, is a frequent feature of ... ...

Abstract	Systemic amyloidosis comprises an uncommon group of disorders caused by the extracellular deposition of misfolded proteins in various organs. Cardiac amyloid deposition, causing an infiltrative/restrictive cardiomyopathy, is a frequent feature of amyloidosis and a major determinant of survival. It may be the presenting feature of the disease or may be identified while investigating a patient presenting with other organ involvement. The need for a high index of suspicion and the critical importance of precise biochemical typing of the amyloid deposits is paramount in light of recent therapeutic advances that can significantly improve prognosis. Most cases of cardiac amyloidosis are of either transthyretin type, which may be acquired in older individuals or inherited in younger patients, or acquired monoclonal immunoglobulin light chain (AL) type. This article aims to review recent developments in diagnosis and management of cardiac amyloidosis.
MeSH term(s)	Amyloidosis ; Cardiomyopathies ; Echocardiography ; Heart/diagnostic imaging ; Heart/physiopathology ; Humans ; Myocardium/pathology ; Prognosis
Language	English
Publishing date	2018-03-06
Publishing country	England
Document type	Journal Article ; Review
ZDB-ID	2048646-7
ISSN	1473-4893 ; 1470-2118
ISSN (online)	1473-4893
ISSN	1470-2118
DOI	10.7861/clinmedicine.18-2-s30
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Article ; Online: Intracardiac melanoma metastases on

Sweni, Shah / Fontana, Marianna / Martinez-Naharro, Ana / Nathan, Malavika

BJR case reports

2019 Volume 5, Issue 3, Page(s) 20180118

Abstract: Cutaneous malignant melanoma is one of the few malignancies that can metastasize to the heart. It is important not to miss cardiac metastases on imaging as they are often clinically asymptomatic, but if present and undiagnosed could lead to significant ... ...

Abstract	Cutaneous malignant melanoma is one of the few malignancies that can metastasize to the heart. It is important not to miss cardiac metastases on imaging as they are often clinically asymptomatic, but if present and undiagnosed could lead to significant cardiac compromise, arrhythmias, congestive heart failure, cardiac tamponade or transient ischaemic attacks. Identifying intracardiac metastases on imaging often requires a multimodality approach as they can evade detection due to cardiac motion artefact; be confused with intracardiac thrombus, or be misinterpreted as a normal/anatomical finding. We present an interesting case report of asymptomatic intracardiac melanoma metastases, initially identified on staging 18-fludeoxyglucose positron emission tomography-CT and eventually confirmed on cardiac MRI. The latter was able to differentiate myocardial metastases from tumour thrombus. We also review the relevant literature.
Language	English
Publishing date	2019-03-28
Publishing country	England
Document type	Journal Article
ISSN	2055-7159
ISSN (online)	2055-7159
DOI	10.1259/bjrcr.20180118
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Article ; Online: Limited utility of Mayo 2012 cardiac staging system for risk stratification of patients with advanced cardiac AL amyloidosis - analysis of a uniformly treated cohort of 1,275 patients.

Khwaja, Jahanzaib / Ravichandran, Sriram / Bomsztyk, Joshua / Cohen, Oliver / Foard, Darren / Martinez-Naharro, Ana / Venneri, Lucia / Fontana, Marianna / Hawkins, Philip N / Gillmore, Julian / Lachmann, Helen J / Mahmood, Shameem / Whelan, Carol / Kirkwood, Amy A / Wechalekar, Ashutosh

Haematologica

2024 Volume 109, Issue 5, Page(s) 1598–1602

MeSH term(s)	Humans ; Immunoglobulin Light-chain Amyloidosis/diagnosis ; Immunoglobulin Light-chain Amyloidosis/mortality ; Immunoglobulin Light-chain Amyloidosis/pathology ; Immunoglobulin Light-chain Amyloidosis/therapy ; Female ; Male ; Aged ; Risk Assessment ; Middle Aged ; Prognosis ; Cohort Studies
Language	English
Publishing date	2024-05-01
Publishing country	Italy
Document type	Letter ; Research Support, Non-U.S. Gov't ; Journal Article
ZDB-ID	2333-4
ISSN	1592-8721 ; 0017-6567 ; 0390-6078
ISSN (online)	1592-8721
ISSN	0017-6567 ; 0390-6078
DOI	10.3324/haematol.2023.284348
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Article ; Online: Prognostic Value of a 6-Minute Walk Test in Patients With Transthyretin Cardiac Amyloidosis.

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

Journal of the American College of Cardiology

2024

Abstract: Background: The 6-minute walk test (6MWT) represents a comprehensive functional assessment that is commonly used in patients with heart failure; however, data are lacking in patients with transthyretin cardiac amyloidosis (ATTR-CA).: Objectives: This ...

Abstract	Background: The 6-minute walk test (6MWT) represents a comprehensive functional assessment that is commonly used in patients with heart failure; however, data are lacking in patients with transthyretin cardiac amyloidosis (ATTR-CA). Objectives: This study aimed to assess the prognostic importance of the 6MWT in patients with ATTR-CA. Methods: A retrospective analysis of patients diagnosed with ATTR-CA at the National Amyloidosis Centre who underwent a baseline 6MWT between 2011 and 2023 identified 2,141 patients, of whom 1,118 had follow-up at 1 year. Results: The median baseline 6MWT distance was 347 m (Q1-Q3: 250-428 m) and analysis by quartiles demonstrated an increased death rate with each distance reduction (deaths per 100 person-years: 6.3 vs 9.2 vs 13.6 vs 19.0; log-rank P < 0.001). A 6MWT distance of <350 m was associated with a 2.2-fold higher risk of mortality (HR: 2.15; 95% CI: 1.85-2.50; P < 0.001), with a similar increased risk across National Amyloidosis Centre disease stages (P for interaction = 0.761) and genotypes (P for interaction = 0.172). An absolute (reduction of >35 m) and relative worsening (reduction of >5%) of 6MWT at 1 year was associated with an increased risk of mortality (HR: 1.80; 95% CI: 1.51-2.15; P < 0.001 and HR: 1.89; 95% CI: 1.59-2.24; P < 0.001, respectively), which was similar across the aforementioned subgroups. When combined with established measures of disease progression (N-terminal pro-B-type natriuretic peptide progression and outpatient diuretic intensification), each incremental increase in progression markers was associated with an increased death rate (deaths per 100 person-years: 7.6 vs 13.9 vs 22.4 vs 32.9; log-rank P < 0.001). Conclusions: The baseline 6MWT distance can refine risk stratification beyond traditional prognosticators. A worsening 6MWT distance can stratify disease progression and, when combined with established markers, identifies patients at the highest risk of mortality.
Language	English
Publishing date	2024-05-07
Publishing country	United States
Document type	Journal Article
ZDB-ID	605507-2
ISSN	1558-3597 ; 0735-1097
ISSN (online)	1558-3597
ISSN	0735-1097
DOI	10.1016/j.jacc.2024.04.011
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Article ; Online: Rare Forms of Cardiac Amyloidosis: Diagnostic Clues and Phenotype in Apo AI and AIV Amyloidosis.

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 / Fontana, Marianna

Circulation. Cardiovascular imaging

2023 Volume 16, Issue 7, Page(s) 523–535

Abstract: Background: Apo AI amyloidosis (AApoAI) and Apo AIV amyloidosis (AApoAIV) are rare but increasingly recognized causes of cardiac amyloidosis (CA). We sought to define the cardiac phenotype in AApoAI and AApoAIV using multimodality imaging.: Methods: ... ...

Abstract	Background: Apo AI amyloidosis (AApoAI) and Apo AIV amyloidosis (AApoAIV) are rare but increasingly recognized causes of cardiac amyloidosis (CA). We sought to define the cardiac phenotype in AApoAI and AApoAIV using multimodality imaging. Methods: We identified all patients with AApoAI and AApoAIV assessed at our center between 2000 and 2021, and 2 cohorts of patients with immunoglobulin light-chain amyloidosis (AL) and transthyretin amyloidosis matched for age, sex, and cardiac involvement. Results: Forty-five patients had AApoAI, 13 (29%) of whom had cardiac involvement, 32 (71%) renal involvement, 28 (62%) splenic involvement, 27 (60%) hepatic involvement, and 7 (16%) laryngeal involvement. AApoAI-CA commonly presented with heart failure (n=8, 62%) or dysphonia (n=7, 54%). The Arg173Pro variant universally caused cardiac and laryngeal involvement (n=7, 100%). AApoAI-CA was associated with right-sided involvement, with a thicker right ventricular free wall (8.6±1.9 versus 6.3±1.3 mm versus 7.7±1.2 mm, Conclusions: Dysphonia, multisystem involvement, or right-sided cardiac disease should raise suspicion of AApoAI-CA. AApoAIV-CA presents most commonly with heart failure and always displays classical CA imaging features, mimicking common forms of CA. Both AApoAI and AApoAIV are associated with a good prognosis and a lower risk of mortality than matched patients with AL-amyloidosis.
MeSH term(s)	Humans ; Apolipoprotein A-I ; Dysphonia/complications ; Prealbumin/genetics ; Amyloid Neuropathies, Familial/complications ; Amyloid Neuropathies, Familial/diagnostic imaging ; Immunoglobulin Light-chain Amyloidosis/diagnostic imaging ; Immunoglobulin Light-chain Amyloidosis/complications ; Heart Failure/complications ; Echocardiography ; Cardiomyopathies/diagnosis
Chemical Substances	Apolipoprotein A-I ; Prealbumin
Language	English
Publishing date	2023-07-11
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	2435045-X
ISSN	1942-0080 ; 1941-9651
ISSN (online)	1942-0080
ISSN	1941-9651
DOI	10.1161/CIRCIMAGING.123.015259
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