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.

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 Cardiac Amyloidosis – An Underdiagnosed Cause of Heart Failure with Preserved Ejection Fraction – Updated Diagnosis and Treatment Options

2021 ◽  
Vol 31 (2) ◽  
pp. 283-302
Author(s):  
Roxana Cristina RIMBAS ◽  
Anca BALINISTEANU ◽  
Alexandra Maria CHITROCEANU ◽  
Dragos VINEREANU
Keyword(s):  
Heart Failure ◽  
Ejection Fraction ◽  
Cardiac Amyloidosis ◽  
Treatment Options ◽  
Preserved Ejection Fraction ◽  
Transthyretin Amyloidosis ◽  
Diagnosis And Prognosis ◽  
Step Algorithm ◽  
Immunoglobulin Light Chain Amyloidosis

Cardiac amyloidosis (CA) still represents a frequently missed cause of heart failure with preserved ejection fraction (HFpEF). In the light of many new and effective therapies for immunoglobulin light chain amyloidosis (AL) and for transthyretin amyloidosis (ATTR), screening for amyloidosis as an important and potentially treatable diagnosis under the HFpEF becomes mandatory. A step-by-step algorithm for CA in HF patients was already provided by the guidelines. This review summarizes the role of all imaging modalities and biomarkers in the diagnosis and prognosis of both subtypes, thealgorithm for diagnosis of CA, and new therapeutic options. It is the first Romanian publication which intends to bring altogether the current recommendations in the diagnosis and management of CA.

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 Amyloidosis Therapy: A Systematic Review

2021 ◽  
Vol 11 (1) ◽  
pp. 10-17
Author(s):  
Franco Iodice ◽  
Marco Di Mauro ◽  
Marco Giuseppe Migliaccio ◽  
Angela Iannuzzi ◽  
Roberta Pacileo ◽  
...  
Keyword(s):  
Heart Failure ◽  
Systematic Review ◽  
Cardiac Amyloidosis ◽  
Restrictive Cardiomyopathy ◽  
Novel Therapies ◽  
Preserved Ejection Fraction ◽  
Cardiac Damage ◽  
Transthyretin Amyloidosis ◽  
To Come ◽  
Almost All

Heart involvement in Cardiac Amyloidosis (CA) results in a worsening of the prognosis in almost all patients with both light-chain (AL) and transthyretin amyloidosis (ATTR). The mainstream CA is a restrictive cardiomyopathy with hypertrophic phenotype at cardiac imaging that clinically leads to heart failure with preserved ejection fraction (HFpEF). An early diagnosis is essential to reduce cardiac damage and to improve the prognosis. Many therapies are available, but most of them have late benefits to cardiac function; for this reason, novel therapies are going to come soon.

scholarly journals Rapid decline in ejection fraction and persistent elevation of troponin associated with cardiac amyloidosis

2020 ◽  
Vol 8 ◽  
pp. 2050313X2092325
Author(s):  
Temidayo Abe ◽  
Eric Y Chang ◽  
Gabrielle De Allie ◽  
Taiwo Ajose ◽  
Chukwuemeka Nwokike ◽  
...  
Keyword(s):  
Heart Failure ◽  
Cardiac Amyloidosis ◽  
Cardiac Biomarkers ◽  
Rapid Decline ◽  
Unique Case ◽  
Transthyretin Amyloidosis ◽  
Progressive Heart Failure ◽  
The Mean ◽  
Persistent Elevation

Cardiac amyloidosis is an increasingly recognized cause of heart failure. It remains underdiagnosed despite a significant morbidity and mortality rate. The mean survival in patients with cardiac amyloidosis is less than 1 year in untreated primary light-chain amyloidosis and less than 4 years in wild-type transthyretin amyloidosis. We report a unique case of a 78-year-old male with transthyretin cardiac amyloidosis, who presented with persistently elevated troponin and progressive heart failure unresponsive to conventional therapy. With this case, we would like to highlight the role of cardiac biomarkers in the early diagnosis of cardiac amyloidosis.

Cardiac Amyloidosis: Overlooked, Underappreciated, and Treatable

2020 ◽  
Vol 71 (1) ◽  
pp. 203-219 ◽  
Author(s):  
Jonah Rubin ◽  
Mathew S. Maurer
Keyword(s):  
Heart Failure ◽  
Cardiac Amyloidosis ◽  
Restrictive Cardiomyopathy ◽  
Preserved Ejection Fraction ◽  
Wild Type ◽  
Immunoglobulin Light Chain ◽  
Future Directions ◽  
Life And Death ◽  
Recent Developments

Cardiac amyloidosis (CA) is an infiltrative and restrictive cardiomyopathy that leads to heart failure, reduced quality of life, and death. The disease has two main subtypes, transthyretin cardiac amyloidosis (ATTR-CA) and immunoglobulin light chain cardiac amyloidosis (AL-CA), characterized by the nature of the infiltrating protein. ATTR-CA is further subdivided into wild-type (ATTRwt-CA) and variant (ATTRv-CA) based on the presence or absence of a mutation in the transthyretin gene. CA is significantly underdiagnosed and increasingly recognized as a cause of heart failure with preserved ejection fraction. Advances in diagnosis that employ nuclear scintigraphy to diagnose ATTR-CA without a biopsy and the emergence of effective treatments, including transthyretin stabilizers and silencers, have changed the landscape of this field and render early and accurate diagnosis critical. This review summarizes the epidemiology, pathophysiology, diagnosis, prognosis, and management of CA with an emphasis on the significance of recent developments and suggested future directions.

Diagnosis of Cardiac Transthyretin Amyloidosis by Technetium-99m Pyrophosphate Heart Scan, Confirmed by Genetic Mutations: Case Report

2021 ◽  
Vol 104 (11) ◽  
pp. 1843-1846
Keyword(s):  
Heart Failure ◽  
Case Report ◽  
Genetic Testing ◽  
Cardiac Amyloidosis ◽  
Abdominal Fat ◽  
Genetic Mutations ◽  
Fat Pad ◽  
Transthyretin Amyloidosis ◽  
Technetium 99M ◽  
Clinically Significant

Amyloid deposition in the myocardium can cause clinically significant heart failure, which is very difficult to diagnose. The present case reported presented a patient with heart failure, with suspected cause of cardiac amyloidosis, but abdominal fat pad and endomyocardial with Congo red stain biopsies were negative. Due to high suspicion of cardiac amyloidosis, a technetium-99m pyrophosphate (Tc-99m PYP) heart scan was done, which was revealed as strongly suggestive for cardiac transthyretin amyloidosis. So, the patient was sent for genetic testing, and a TTR gene mutation [c.148G>A (p.Val50Met)] was found. Keywords: Cardiac amyloidosis; Endomyocardial biopsy (EMB); Technetium-99m pyrophosphate (Tc-99m PYP) heart scan; Transthyretin amyloidosis (TTR)

scholarly journals Strange case of biventricular heart failure

2021 ◽  
Vol 14 (1) ◽  
pp. e239658
Author(s):  
Carloalberto Biolè ◽  
Matteo Bianco ◽  
Antonella Parente ◽  
Laura Montagna
Keyword(s):  
Heart Failure ◽  
Pulmonary Embolism ◽  
Cardiac Amyloidosis ◽  
Left Ventricular ◽  
Pulmonary Congestion ◽  
Transthyretin Amyloidosis ◽  
X Ray ◽  
Infiltrative Cardiomyopathy ◽  
Mild Reduction ◽  
Chest X Ray

Acute heart failure (HF) is commonly caused by a cardiomyopathy with one or more precipitating factor. Here, a case in which a cardiomyopathy is precipitated by pulmonary embolism (PE). A 77-year-old man is admitted for breathlessness and leg swelling. A mild reduction of left ventricular (LV) ejection fraction is found, with moderately increased LV wall thickness and pulmonary hypertension; clinical examination revealed signs of congestion with bilateral leg swelling, and mild signs of left HF with the absence of pulmonary congestion on chest X-ray. The ECG showed Mobitz I second-degree atrioventricular block. The clinical scenario led us to the diagnosis of infiltrative cardiomyopathy due to cardiac amyloidosis (CA) precipitated by PE. Pulmonary embolism is an overlooked precipitant of HF and can be the first manifestation of an underlying misdiagnosed cardiomyopathy, especially CA. 3,3-Diphosphono-1,2-propanodicarboxylic acid scan is a cornerstone in the diagnosis of Transthyretin amyloidosis (ATTR) cardiac amyloidosis.

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