scholarly journals Transthyretin amyloid cardiomyopathy: The emerging role of cardiac amyloid imaging

2021 ◽  
Vol 50 (7) ◽  
pp. 566-571
Author(s):  
Sarah Ming Li Tan ◽  
Yoke Ching Lim ◽  
Ping Chai ◽  
Lenith Tai Jit Cheng ◽  
Ching Hui Sia ◽  
...  
Keyword(s):  
Amyloid Imaging ◽  
Cardiac Amyloid ◽  
Amyloid Cardiomyopathy

Abstract 19078: Early Predictors of Survival to Heart Transplantation in Patients With Amyloid Light-chain Cardiomyopathy

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Johannes Steiner ◽  
Van Selby ◽  
Karla Verkouw ◽  
Jana Svetlichnaya ◽  
Doreen Defaria Yeh ◽  
...  
Keyword(s):  
Cardiac Output ◽  
Heart Transplantation ◽  
Right Ventricular ◽  
Light Chain ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Cell Transplant ◽  
Stroke Work ◽  
Cardiac Amyloid ◽  
Amyloid Cardiomyopathy

Background: Amyloid cardiomyopathy progresses more rapidly and has different manifestations than more common cardiomyopathies. Nevertheless, survival of selected cardiac amyloid patients after orthotopic heart transplantation (OHT) followed by autologous stem cell transplant is similar to other restrictive cardiomyopathies. The importance of clinical presentation and pre-transplantation characteristics on outcome in patients with AL amyloid cardiomyopathy evaluated for heart transplantation is still not well defined. Methods: The impact of echocardiographic, hemodynamic, and clinical parameters on outcome in 46 patients evaluated for OHT and enrolled in the International Consortium for Cardiac Amyloid Transplant (iCCAT) database was studied. Cox proportional hazards models of time to death were used. End point was death after transplant evaluation censored at transplant. Results: The median age at the time of transplant evaluated was 57.6 (+/- 9.6) years, and the mean time from presentation to listing was 27 (+/- 26) days. 19 (41%) patients underwent OHT after an average wait time of 72 days. Univariate risk factors associated with death after initial evaluation were cardiac output (p=0.012), right ventricular stroke work index (p=0.033), left ventricular end diastolic dimension (p=0.024), left ventricular outflow tract velocity time integral (p=0.019), mean arterial pressures (p=0.005), NT pro-BNP (p=0.007), presence of pleural effusion (p=0.005), as well as elevated kappa and lambda serum free light chain concentrations (p=0.004). Whereas left ventricular ejection fraction did not correlate with death after evaluation, increased right ventricular wall thickness remained a strong mortality predictor in a multivariate model with light chain difference and cardiac output (HR 1.6 for every 1 mm increase in thickness, p=0.017). Conclusions: Pleural involvement, high light chain burden, and right ventricular infiltration predicted death while awaiting a donor organ and may be markers of more systemic disease. Ultimately, this could affect transplant candidate selection and allocation strategies.

scholarly journals The Role of Cardiovascular Magnetic Resonance Imaging in Heart Failure

2016 ◽  
pp. 115 ◽  
Author(s):  
Mark A Peterzan ◽  
Oliver J Rider ◽  
Lisa J Anderson
Keyword(s):  
Magnetic Resonance Imaging ◽  
Heart Failure ◽  
Cardiovascular Magnetic Resonance ◽  
Magnetic Resonance ◽  
Cardiovascular Magnetic Resonance Imaging ◽  
Resonance Imaging ◽  
Cardiac Amyloid ◽  
Patients With Heart Failure ◽  
Established Role

Cardiovascular imaging is key for the assessment of patients with heart failure. Today, cardiovascular magnetic resonance imaging plays an established role in the assessment of patients with suspected and confirmed heart failure syndromes, in particular identifying aetiology. Its role in informing prognosis and guiding decisions around therapy are evolving. Key strengths include its accuracy; reproducibility; unrestricted field of view; lack of radiation; multiple abilities to characterise myocardial tissue, thrombus and scar; as well as unparalleled assessment of left and right ventricular volumes. T2* has an established role in the assessment and follow-up of iron overload cardiomyopathy and a role for T1 in specific therapies for cardiac amyloid and Anderson–Fabry disease is emerging.

scholarly journals Cardiac Amyloid Imaging with 18F-Florbetaben PET: A Pilot Study

2016 ◽  
Vol 57 (11) ◽  
pp. 1733-1739 ◽  
Author(s):  
W. P. Law ◽  
W. Y. S. Wang ◽  
P. T. Moore ◽  
P. N. Mollee ◽  
A. C. T. Ng
Keyword(s):  
Pilot Study ◽  
Amyloid Imaging ◽  
Cardiac Amyloid

Cardiac amyloid imaging with 18F-florbetaben positron emission tomography: a pilot study

Amyloid ◽  
2017 ◽  
Vol 24 (sup1) ◽  
pp. 162-162 ◽  
Author(s):  
W. Phillip Law ◽  
William Wang ◽  
Peter Moore ◽  
Peter Mollee ◽  
Arnold Ng
Keyword(s):  
Positron Emission Tomography ◽  
Pilot Study ◽  
Amyloid Imaging ◽  
Emission Tomography ◽  
Cardiac Amyloid ◽  
Positron Emission

scholarly journals Recurrent Syncope, a Clue in Amyloid Cardiomyopathy

2018 ◽  
Vol 2018 ◽  
pp. 1-6
Author(s):  
Julian A. Marin-Acevedo ◽  
Catalina Sanchez-Alvarez ◽  
Ali A. Alsaad ◽  
Ricardo J. Pagán
Keyword(s):  
Heart Failure ◽  
Decision Making ◽  
Cardiac Amyloidosis ◽  
Clinical Manifestations ◽  
Medical Team ◽  
Shared Decision ◽  
Amyloid Cardiomyopathy ◽  
Myocardial Thickening ◽  
Infiltrative Cardiomyopathies

Infiltrative cardiomyopathies include a variety of disorders that lead to myocardial thickening resulting in a constellation of clinical manifestations and eventually heart failure that could be the first clue to reach the diagnosis. Among the more described infiltrative diseases of the heart is amyloid cardiomyopathy. The disease usually presents with subtle, nonspecific symptoms. Herein, we illustrate a case of recurrent syncope as the initial presenting symptom for systemic amyloid with polyneuropathy and cardiomyopathy as a cause of syncope. The article illustrates the role of advanced cardiac imaging in the diagnosis of the disease with a focused literature review. We also highlight the role of early, shared decision-making between patient, family, and medical team in the management of cardiac amyloidosis.

Cardiac amyloid imaging (DPD scans)

2021 ◽  
Author(s):  
Federico Caobelli ◽  
Cristina E. Popescu
Keyword(s):  
Amyloid Imaging ◽  
Cardiac Amyloid

scholarly journals A Case of Early Hereditary Transthyretin Amyloid Cardiomyopathy Recognition With Genetic Screening: A Case Report

2022 ◽  
Vol 13 ◽  
pp. 215013192110626
Author(s):  
Michel Juarez ◽  
Gaspar Del Rio-Pertuz ◽  
Kanak Parmar ◽  
Melanie C. Bois ◽  
Scott Shurmur ◽  
...  
Keyword(s):  
Heart Failure ◽  
African American ◽  
Genetic Screening ◽  
Signs And Symptoms ◽  
Restrictive Cardiomyopathy ◽  
Cardiac Amyloid ◽  
Carpal Tunnel Surgery ◽  
History Of ◽  
Amyloid Cardiomyopathy ◽  
Men Elderly

Background: Transthyretin amyloid cardiomyopathy (ATTR-CM) is one of the most common types of cardiac amyloidosis. Amyloid cardiomyopathy more commonly affects men, elderly, and 3% to 4% of the African-American population. ATTR-CM suspicion and diagnosis is challenging; however, awareness of the disease is increasing, and best practices to identify it are being proposed. The approach to suspected cases of ATTR-CM relies on the presence of heart failure, red flag signs and symptoms, and age >65 or >70 for men and women respectively. Little is known about cases when it presents in early ages. Case: We report a 62-year-old African American male with past medical history of hyperlipidemia, prostate cancer, hypertension, bilateral carpal tunnel surgery that had debuted with a cardiac arrhythmia at age 55 and was diagnosed with heart failure several years later. Restrictive cardiomyopathy was suspected, and genetic screening was sent for ATTRm which confirmed a pathogenic trasnthyretin gene mutation. Endomyocardial biopsy was performed which confirmed cardiac amyloid deposition. Discussion: ATTR-CM is a rare disease with an increasing prevalence. Cases with out of proportion signs and symptoms of heart failure with preserved ejection fractions should raise the suspicion of ATTR-CM despite age.

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