Diagnosis and treatment of cardiac amyloidosis: position statement of the German Cardiac Society (DGK)

AbstractSystemic forms of amyloidosis affecting the heart are mostly light-chain (AL) and transthyretin (ATTR) amyloidoses. The latter is caused by deposition of misfolded transthyretin, either in wild-type (ATTRwt) or mutant (ATTRv) conformation. For diagnostics, specific serum biomarkers and modern non-invasive imaging techniques, such as cardiovascular magnetic resonance imaging (CMR) and scintigraphic methods, are available today. These imaging techniques do not only complement conventional echocardiography, but also allow for accurate assessment of the extent of cardiac involvement, in addition to diagnosing cardiac amyloidosis. Endomyocardial biopsy still plays a major role in the histopathological diagnosis and subtyping of cardiac amyloidosis. The main objective of the diagnostic algorithm outlined in this position statement is to detect cardiac amyloidosis as reliably and early as possible, to accurately determine its extent, and to reliably identify the underlying subtype of amyloidosis, thereby enabling subsequent targeted treatment.

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From Clinical Clues to Final Diagnosis: The Return of Detective Work to Clinical Medicine in Cardiac Amyloidosis

Frontiers in Cardiovascular Medicine ◽

10.3389/fcvm.2021.644508 ◽

2021 ◽

Vol 8 ◽

Author(s):

Hani Sabbour ◽

Khwaja Yousuf Hasan ◽

Firas Al Badarin ◽

Haluk Alibazoglu ◽

Andrew L. Rivard ◽

...

Keyword(s):

Cardiac Amyloidosis ◽

Clinical Medicine ◽

Imaging Techniques ◽

Diagnostic Algorithm ◽

Signs And Symptoms ◽

Final Diagnosis ◽

Diagnostic Tools ◽

Case Examples ◽

Non Invasive ◽

High Index Of Suspicion

Cardiac amyloidosis is frequently misdiagnosed, denying patients the opportunity for timely and appropriate management of the disease. The purpose of this review and case studies is to raise awareness of the diagnostic “red flags” associated with cardiac amyloidosis and the currently available non-invasive strategies for diagnosis. The review focuses on the identification of one of the two main types of cardiac amyloidosis, transthyretin amyloid cardiomyopathy, and non-invasive tools to distinguish this from light-chain amyloidosis. A diagnostic algorithm centered around the use of non-invasive imaging and laboratory analysis is presented. The algorithm generates four differential diagnoses for patients presenting with signs and symptoms consistent with cardiac amyloidosis. Case examples are presented, representing the four potential outcomes of diagnosis using the algorithm. The review provides a guide on how to recognize the often-overlooked presentations of this disease in clinical practice. Non-invasive imaging techniques and diagnostic tools that do not require the involvement of a specialty center have allowed for the improved diagnosis of cardiac amyloidosis. Timely diagnosis of this life-threatening disease is essential for optimal management and it is imperative that clinicians have a high index of suspicion for patients presenting with “red flag” symptoms.

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Cardiac amyloidosis: a review of the literature and a practical approach for the clinicians

Italian Journal of Medicine ◽

10.4081/itjm.2019.1149 ◽

2019 ◽

Vol 13 (2) ◽

pp. 73-90 ◽

Cited By ~ 1

Author(s):

Annalisa Angelini ◽

Francesca Zanco ◽

Chiara Castellani ◽

Andrea Di Francesco ◽

Mila Della Barbera ◽

...

Keyword(s):

Cardiac Amyloidosis ◽

Imaging Techniques ◽

Systemic Disease ◽

Treatment Strategies ◽

Diagnostic Methods ◽

Review Of The Literature ◽

Cardiac Tissues ◽

Non Invasive ◽

Precursor Proteins ◽

Recent Treatment

Amyloidosis is a group of progressive and devastating disorders resulting from misfolded proteins extracellular deposition into tissues. When deposition of fibrils occurs in cardiac tissues, this systemic disease can lead to a very poor prognosis. In this review, we focused on the most common types of cardiac amyloidosis and their treatments. Early diagnosis remains critically important, and here we reviewed the diagnostic methods adopted starting from the non-invasive imaging techniques to more invasive approaches, and the typing of precursor proteins. Typing the different misfolding proteins is mandatory since therapy differs accordingly and thus guiding therapy. We highlighted the most updated and recent treatment strategies to cure amyloidosis.

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Strain imaging in right ventricle assessment

Oxford Textbook of Advanced Critical Care Echocardiography ◽

10.1093/med/9780198749288.003.0027 ◽

2020 ◽

pp. 343-350

Author(s):

Sam Orde

Keyword(s):

Right Ventricle ◽

Cardiac Dysfunction ◽

Imaging Techniques ◽

Cardiac Mechanics ◽

Critically Ill Patient ◽

Non Invasive ◽

Imaging Tool ◽

Right Ventricle Free Wall ◽

The Right ◽

Conventional Echocardiography

The right ventricle is now recognized as being integral to cardiac mechanics and analysis of its function is an essential part of any echocardiogram performed in the critically ill patient. However, it has a complex triangular conical shape and is located retrosternally making it difficult to image. Unlike the left ventricle (LV) with its myocardial fibres in many different directions, the right ventricle (RV) has a predominance of longitudinal fibres with most of its movement being in a basal to apex direction. This makes it sensitive to analysis with speckle tracking echocardiography analysis of longitudinal strain: commonly reported as right ventricle free wall strain. Strain is a measure of relative myocardial deformation analysed through tracking of the speckles that make up the myocardium on the two-dimensional B-mode image. It is a postprocessing imaging tool and experience in echocardiography is required before tackling this form of assessment. Strain is sensitive, reproducible, angle independent, not prone to translational error like other conventional echocardiography tools and most importantly can recognize cardiac dysfunction and mechanics that cannot be described by other non-invasive imaging techniques. No echocardiography parameter used to assess right ventricle function is perfect, including right ventricle strain assessment. However its advantages are witnessed by the fact that it has entered clinical practice (exclusively to cardiology departments at this stage) in many larger centres around the world.

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Multimodality imaging for the diagnosis of infiltrative cardiomyopathies

Heart ◽

10.1136/heartjnl-2020-318001 ◽

2021 ◽

pp. heartjnl-2020-318001

Author(s):

Mahesh K Vidula ◽

Paco E Bravo

Keyword(s):

Iron Overload ◽

Cardiac Amyloidosis ◽

Cardiac Sarcoidosis ◽

Multimodality Imaging ◽

Imaging Techniques ◽

Nuclear Imaging ◽

Non Invasive ◽

Iron Overload Cardiomyopathy ◽

Appropriate Treatment ◽

Infiltrative Cardiomyopathies

Infiltrative cardiomyopathies result from the deposition or anomalous storage of specific substances in the heart, leading to impaired cardiac function and heart failure. In this review, we describe the utility of a variety of imaging modalities for the diagnosis of infiltrative cardiomyopathies and provide algorithms for clinicians to use to evaluate patients with these disorders. We have divided infiltrative cardiomyopathies into two different categories: (1) infiltrative cardiomyopathies characterised by increased wall thickness (eg, cardiac amyloidosis and Anderson-Fabry disease (AFD)) and (2) infiltrative cardiomyopathies that can mimic ischaemic or dilated cardiomyopathies (eg, cardiac sarcoidosis (CS) and iron overload cardiomyopathy). Echocardiography is the first modality of choice for the evaluation of cardiomyopathies in either category, and the differential can be narrowed using cardiac magnetic resonance (CMR) and nuclear imaging techniques. The diagnosis of cardiac amyloidosis is supported with key findings seen on echocardiography, CMR and nuclear imaging, whereas AFD can be suggested by unique features on CMR. CMR and nuclear imaging are also important modalities for the diagnosis of CS, while iron overload cardiomyopathy is mostly diagnosed using tissue characterisation on CMR. Overall, multimodality imaging is necessary for the accurate non-invasive diagnosis of infiltrative cardiomyopathies, which is important to ensure appropriate treatment and prognostication.

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IMAGING TECHNIQUES IN THE DIAGNOSIS OF PULMONARY HYPERTENSION

Wiadomości Lekarskie ◽

10.36740/wlek202009111 ◽

2020 ◽

Vol 73 (9) ◽

pp. 1867-1869

Author(s):

Weronika Topyła ◽

Michał Tomaszewski ◽

Agnieszka Wojtkowska ◽

Sylwia Łukasik ◽

Andrzej Wysokiński ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Clinical Symptoms ◽

Imaging Techniques ◽

Diagnostic Algorithm ◽

Right Heart Catheterization ◽

Diagnostic Process ◽

Heart Catheterization ◽

Hypertension Detection ◽

Pulmonary Hemodynamics ◽

Non Invasive

Introduction: Pulmonary hypertension is defined as being a haemodynamic state, wherein the mean pulmonary artery pressure measured during right heart catheterization is equal or greater than 25 mmHg. As a result, right ventricular heart failure develops and clinical symptoms such as dyspnea, fatigue, weakness, angina and fainting occur. The aim: To highlight the role of imaging techniques in diagnostic process for pulmonary hypertension. Review and Disscusion: The diagnosis of pulmonary hypertension is multistage and often requires a number of studies. Currently, imaging techniques play a significant role in the diagnostic algorithm for pulmonary hypertension due to the fact that they are non-invasive and readily available, and many of their parameters are closely related to pulmonary hemodynamics. Conclusions: The diagnosis of pulmonary hypertension requires a multistep approach and a number of imaging studies. The suspicion of the disease is based on medical history, clinical symptoms and chest radiogram. Echocardiography plays a crucial role in pulmonary hypertension detection. A computed tomography and cardiac magnetic resonanceare valuable methods in determining the cause of suspected or confirmed pulmonary hypertension.

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

Current Pharmaceutical Design ◽

10.2174/1381612826666200813133557 ◽

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.

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Methods and clinical applications in nuclear cardiology: a position statement

Nuklearmedizin ◽

10.1055/s-0038-1623996 ◽

2002 ◽

Vol 41 (01) ◽

pp. 3-13 ◽

Cited By ~ 4

Author(s):

M. Schäfers

Keyword(s):

Nuclear Cardiology ◽

Clinical Medicine ◽

Imaging Techniques ◽

Cost Savings ◽

Cost Benefit ◽

Position Statement ◽

Electron Beam Tomography ◽

Partial Functions ◽

Cost Benefit Ratio ◽

Medicine Today

SummaryNuclear cardiological procedures have paved the way for non-invasive diagnostics of various partial functions of the heart. Many of these functions cannot be visualised for diagnosis by any other method (e. g. innervation). These techniques supplement morphological diagnosis with regard to treatment planning and monitoring. Furthermore, they possess considerable prognostic relevance, an increasingly important issue in clinical medicine today, not least in view of the cost-benefit ratio.Our current understanding shows that effective, targeted nuclear cardiology diagnosis – in particular for high-risk patients – can contribute toward cost savings while improving the quality of diagnostic and therapeutic measures.In the future, nuclear cardiology will have to withstand mounting competition from other imaging techniques (magnetic resonance imaging, electron beam tomography, multislice computed tomography). The continuing development of these methods increasingly enables measurement of functional aspects of the heart. Nuclear radiology methods will probably develop in the direction of molecular imaging.

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Integration Between Computed Tomography and Nuclear Medicine for Non-invasive Assessment of Coronary Anatomy and Myocardial Perfusion

European Cardiology Review ◽

10.15420/ecr.2012.5.2.15 ◽

2009 ◽

Vol 5 (2) ◽

pp. 15

Author(s):

Wanda Acampa ◽

Mario Petretta ◽

Carmela Nappi ◽

Alberto Cuocolo ◽

◽

...

Keyword(s):

Computed Tomography ◽

Coronary Heart Disease ◽

Blood Flow ◽

Heart Disease ◽

Myocardial Perfusion ◽

Myocardial Blood Flow ◽

Imaging Techniques ◽

Emission Computed Tomography ◽

Coronary Anatomy ◽

Non Invasive

Many non-invasive imaging techniques are available for the evaluation of patients with known or suspected coronary heart disease. Among these, computed-tomography-based techniques allow the quantification of coronary atherosclerotic calcium and non-invasive imaging of coronary arteries, whereas nuclear cardiology is the most widely used non-invasive approach for the assessment of myocardial perfusion. The available single-photon-emission computed tomography flow agents are characterised by a cardiac uptake proportional to myocardial blood flow. In addition, different positron emission tomography tracers may be used for the quantitative measurement of myocardial blood flow and coronary flow reserve. Extensive research is being performed in the development of non-invasive coronary angiography and myocardial perfusion imaging using cardiac magnetic resonance. Finally, new multimodality imaging systems have recently been developed bringing together anatomical and functional information. This article provides a description of the available non-invasive imaging techniques in the assessment of coronary anatomy and myocardial perfusion in patients with known or suspected coronary heart disease.

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