Imaging Techniques As An Aid In The Early Detection Of Cardiac Amyloidosis

2020 ◽  
Vol 26 ◽  
Author(s):  
M.F. Santarelli ◽  
M. Scipioni ◽  
D. Genovesi ◽  
A. Giorgetti ◽  
P. Marzullo ◽  
...  
Keyword(s):  
Early Detection ◽  
Early Diagnosis ◽  
Cardiac Amyloidosis ◽  
Imaging Techniques ◽  
Myocardial Biopsy ◽  
Immunoglobulin Light Chain ◽  
Transthyretin Amyloidosis ◽  
Non Invasive ◽  
Immunoglobulin Light Chain Amyloidosis

: The idea that performing a proper succession of imaging tests and techniques allows an accurate and early diagnosis of cardiac amyloidosis, avoiding the need to perform myocardial biopsy, is becoming increasingly popular. Furthermore, being imaging techniques non-invasive, it is possible to perform the follow-up of the pathology through repeated image acquisitions. In the present review, the various innovative imaging methodologies are presented, and it is discussed how they have been applied for early diagnosis of cardiac amyloidosis (CA), also to distinguish the two most frequent subtypes in CA: immunoglobulin light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR); this allows to perform the therapy in a targeted and rapid manner.

scholarly journals Cardiac Care of Patients with Cardiac Amyloidosis

2020 ◽  
Vol 143 (4) ◽  
pp. 343-351
Author(s):  
Osnat Itzhaki Ben Zadok ◽  
Ran Kornowski
Keyword(s):  
Heart Failure ◽  
Cardiac Amyloidosis ◽  
Management Strategies ◽  
Treatment Strategies ◽  
Multidisciplinary Care ◽  
Cardiac Care ◽  
Immunoglobulin Light Chain ◽  
Transthyretin Amyloidosis ◽  
Immunoglobulin Light Chain Amyloidosis ◽  
Specific Management

Cardiac amyloidosis, the majority of cases of which are due to immunoglobulin light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR), affects different aspects of the heart and cardiovascular system. Amyloid-induced cardiomyopathy, clinically manifesting with heart failure and electrophysiological abnormalities, has distinct characteristics compared to non-amyloid cardiomyopathies. Accordingly, specific management strategies are required. This paper will review the cardiovascular manifestations of patients with cardiac amyloidosis and their suggested treatment strategies, emphasizing the importance of multidisciplinary care.

Keys to early diagnosis of cardiac amyloidosis: red flags from clinical, laboratory and imaging findings

2019 ◽  
Vol 27 (17) ◽  
pp. 1806-1815 ◽  
Author(s):  
Giuseppe Vergaro ◽  
Alberto Aimo ◽  
Andrea Barison ◽  
Dario Genovesi ◽  
Gabriele Buda ◽  
...  
Keyword(s):  
Early Diagnosis ◽  
Light Chain ◽  
Cardiac Amyloidosis ◽  
Cardiac Involvement ◽  
Clinical Laboratory ◽  
Early Recognition ◽  
Strong Predictor ◽  
Red Flags ◽  
Immunoglobulin Light Chain ◽  
Transthyretin Amyloidosis

Cardiac involvement in systemic amyloidosis, due either to immunoglobulin light-chain or transthyretin amyloidosis, influences clinical presentation and is a strong predictor of unfavourable outcome. Until recently considered as a rare, incurable disease, cardiac amyloidosis, is still mis/underdiagnosed, although treatments effective in improving patient survival are now available for both subtypes, including chemotherapy regimens for immunoglobulin light-chain amyloidosis and tetramer stabiliser for transthyretin amyloidosis. Achieving a timely diagnosis allows initiating life-saving therapies and requires the early recognition of clinical, laboratory and imaging signs of cardiac involvement, some of them may be apparent well before the disease becomes clinically manifest. Given the systemic nature of amyloidosis, a close interaction among experts in multiple specialties is also required, including cardiologists, nephrologists, haematologists, neurologists, radiologists, nuclear medicine specialists and internists. As an increased awareness about disease presentation is required to ameliorate diagnostic performance, we aim to provide the clinician with a guide to the screening and early diagnosis of cardiac amyloidosis, and to review the clinical, biohumoral and instrumental ‘red flags’ that should raise the suspicion of cardiac amyloidosis.

scholarly journals Cardiac Amyloidosis with Discordant QRS Voltage between Frontal and Precordial Leads

Medicina ◽  
2021 ◽  
Vol 57 (7) ◽  
pp. 660
Author(s):  
Csilla-Andrea Eötvös ◽  
Roxana-Daiana Lazar ◽  
Iulia-Georgiana Zehan ◽  
Erna-Brigitta Lévay-Hail ◽  
Giorgia Pastiu ◽  
...  
Keyword(s):  
Left Ventricular Hypertrophy ◽  
Cardiac Amyloidosis ◽  
Ventricular Hypertrophy ◽  
Low Voltage ◽  
Disease Process ◽  
Left Ventricular ◽  
Clinical Suspicion ◽  
Immunoglobulin Light Chain ◽  
Different Types

Among the different types, immunoglobulin light chain (AL) cardiac amyloidosis is associated with the highest morbidity and mortality. The outcome, however, is significantly better when an early diagnosis is made and treatment initiated promptly. We present a case of cardiac amyloidosis with left ventricular hypertrophy criteria on the electrocardiogram. After 9 months of follow-up, the patient developed low voltage in the limb leads, while still maintaining the Cornell criteria for left ventricular hypertrophy as well. The relative apical sparing by the disease process, as well as decreased cancellation of the opposing left ventricular walls could be responsible for this phenomenon. The discordance between the voltage in the frontal leads and precordial leads, when present in conjunction with other findings, may be helpful in raising the clinical suspicion of cardiac amyloidosis.

scholarly journals Diagnosis of Cardiac Amyloidosis Using Non-Invasive Technics

2021 ◽  
Author(s):  
Eva Strickler ◽  
Ernest Tsiaze ◽  
Gerrit Hellige ◽  
Dominik Zumstein ◽  
Dominik Waldmeier ◽  
...  
Keyword(s):  
Cardiac Amyloidosis ◽  
Al Amyloidosis ◽  
Definite Diagnosis ◽  
Diagnostic Features ◽  
Transthyretin Amyloidosis ◽  
Cardiac Amyloid ◽  
Non Invasive ◽  
Amyloid Accumulation ◽  
Clinically Significant ◽  
Multiorgan Disease

Amyloidosis is a rare multiorgan disease defined by a process of irreversible, extracellular accumulation of fibrillar proteins in the tissues, including the heart. Cardiac involvement is seen in most forms of amyloidosis, but it is frequently present and clinically significant in light chain (AL)-amyloidosis as well as transthyretin amyloidosis (ATTR). Cardiac amyloid accumulation leads to a restrictive filling pattern, which must be differentiated from other forms of restrictive and hypertrophic cardiomyopathies due to consequences for the treatment. Evolving knowledge of the disease has led to a definite diagnosis of the cardiac amyloidosis (CA) using non-invasive and low-risk diagnostic features, such as scintigraphy (gamma scan) and cardiovascular magnetic resonance (CMR) imaging using late gadolinium enhancement (LGE) and T1 mapping technics. The availability and diagnostic accuracy of these technics has reduced the need for cardiac biopsy. In the following chapter, we will describe common types of CA, the basic concepts, and updates of non-invasive diagnostic features.

Cardiac amyloidosis

ESC CardioMed ◽  
2018 ◽  
pp. 1545-1549
Author(s):  
Martha Grogan
Keyword(s):  
Heart Failure ◽  
Median Survival ◽  
Cardiac Amyloidosis ◽  
Beta Blockers ◽  
Angiotensin Converting Enzyme Inhibitors ◽  
Wild Type ◽  
Transthyretin Amyloidosis ◽  
Converting Enzyme Inhibitors ◽  
Right Ventricular Wall ◽  
Immunoglobulin Light Chain Amyloidosis

Cardiac amyloidosis is an important cause of heart failure and cardiac arrhythmias, yet cardiologists often miss the diagnosis. Immunoglobulin light-chain amyloidosis (AL) is relatively rare, but likely underdiagnosed. The median survival of untreated patients with cardiac AL is 6 months after the onset of heart failure, highlighting the importance of early diagnosis. Wild-type transthyretin amyloidosis (ATTR) is increasingly recognized, especially in males over the age of 60 years. Although the clinical course of wild-type ATTR is more indolent, the median survival is approximately 3.5 years from diagnosis. Typical echocardiographic findings of increased left and right ventricular wall thickness, diastolic dysfunction, and pericardial effusion may suggest cardiac amyloidosis, along with abnormal delayed gadolinium enhancement and difficulty nulling the myocardium on cardiac magnetic resonance imaging. For AL, a tissue diagnosis is required. In contrast, ATTR may be diagnosed non-invasively with grade 2/3 uptake by nuclear scintigraphy in the absence of a monoclonal protein. Treatment of cardiac amyloidosis is entirely dependent on the type of amyloid and is directed at the underlying precursor protein or disrupting existing deposits. Cardiac care is supportive and challenging. Standard heart failure medications such as beta blockers and angiotensin-converting enzyme inhibitors are not routinely indicated and often cause haemodynamic deterioration. Outcomes of source-directed therapy for AL are improving and several clinical trials of treatment for ATTR are ongoing.

scholarly journals Standardized Protocol for Hemodialysis Vascular Access Assessment: The Role of Ultrasound and ColorDoppler

2018 ◽  
Vol 45 (1-3) ◽  
pp. 260-269 ◽  
Author(s):  
Federico Nalesso ◽  
Francesco Garzotto ◽  
Ilaria Petrucci ◽  
Sara Samoni ◽  
Grazia Maria Virzì ◽  
...  
Keyword(s):  
Early Diagnosis ◽  
Vascular Access ◽  
Hemodynamic Parameters ◽  
Non Invasive ◽  
Standardized Protocol ◽  
Hemodialysis Vascular Access ◽  
Simple Step ◽  
Artery Flow

Introduction: Ultrasound and colorDoppler technique, which is relatively inexpensive, rapid, non-invasive and repeatable is a powerful tool used for early diagnosis of vascular access (VA) complications in hemodialysis patients. To date a standard and widely comprehensible echocolorDoppler (ECD) protocol is not available. Materials and Methods: A simple step-by-step protocol based on anatomical and hemodynamic parameters of VA has been developed during a 3-years VA ECD follow-up. It consists of an ECD study scheme. The algorithm created involves the calculation of brachial artery flow, description of artero-venous and/or graft-vascular anastomosis and efferent vessel and/or graft. Results: The algorithm allows to formulate a medical report that takes into account both anatomic and hemodynamic parameters of the VA. Reduction of complications and the prevention of chronic complications as well as the early detection of acute problems were achieved. Discussion and Conclusion: The creation of a step-by-step protocol may simplify the multidisciplinary management of VA, its monitoring and the early diagnosis of its complications.

scholarly journals AL-amyloidosis with cardiac involvement. Diagnostic capabilities of non-invasive methods

2021 ◽  
Vol 93 (4) ◽  
pp. 487-496
Author(s):  
Alexandra Ya. Gudkova ◽  
Sergei V. Lapekin ◽  
Tinatin G. Bezhanishvili ◽  
Maria A. Trukshina ◽  
Victoria G. Davydova ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Cardiac Amyloidosis ◽  
Cardiac Involvement ◽  
Clinical Course ◽  
Al Amyloidosis ◽  
Transthyretin Amyloidosis ◽  
Non Invasive ◽  
Amyloid Cardiomyopathy ◽  
Diagnostic Capabilities ◽  
Invasive Diagnostics

There are presented the literature data and a description of the clinical course of the disease in isolated/predominant cardiac amyloidosis. Amyloid cardiomyopathy is the most common phenocopy of hypertrophic cardiomyopathy. The modern possibilities of non-invasive diagnostics using osteoscintigraphy for the differential diagnosis between amyloid cardiomyopathy caused by AL- and transthyretin amyloidosis are described in detail.

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