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Cardiac Hypertrophy, Heart Failure and Cardiomyopathy
Published in Mary N. Sheppard, Practical Cardiovascular Pathology, 2022
The next most common amyloid deposition occurs in both the atria and the ventricles. The amyloid may be immunoglobulin in origin (amyloid AL); secondary to chronic infection or myeloma where the amyloid is a part of the acute phase serum amyloid A (amyloid AA); or familial forms in which transthyretin proteins are deposited. Cardiac involvement occurs in one third of cases of immunoglobulin light chain (AL) variant amyloid, and is the cause of death in up to half of these patients. Myocardial involvement in transthyretin (TTR) amyloidosis varies according to the specific transthyretin mutation, and it is rare in secondary (AA) amyloidosis. In all these forms, extra-cardiac deposits are usually widespread by the time cardiac involvement is apparent. Cardiac amyloidosis particularly in elderly males, with wild-type transthyretin amyloid (ATTR) deposits is seen in the heart at autopsy in 25% of patients aged 85 and over. So, it follows that cardiac amyloidosis is likely to coexist in a significant proportion of elderly patients with aortic stenosis. We have incomplete knowledge about amyloid prevalence in healthy ageing. It is suggested that AS primes the myocardium for amyloid deposition. There are hints that AS-amyloid is not like ‘typical’ cardiac amyloid so we should not assume that the impact of amyloid-specific therapies such as tafamidis, patisiran and inotersen will be the same as in lone amyloid.
Beyond the Valve and into the Muscle: A Review of Coexisting Aortic Stenosis and Transthyretin Cardiac Amyloidosis
Published in Structural Heart, 2019
Hannah Rosenblum, David L. Narotsky, Nadira Hamid, Rebecca T. Hahn, Susheel Kodali, Tamim Nazif, Omar K. Khalique, Sabahat Bokhari, Mathew S. Maurer, Adam Castaño
Among studies that included myocardial sampling in patients undergoing aortic valve replacement for AS, the prevalence of ATTR-CA has ranged from 4% to 29%. The highest prevalence of ATTR-CA was found in a small, postmortem analysis of 17 subjects who had undergone transcatheter aortic valve replacement.6 In contrast, in a study of 146 patients who underwent surgical aortic valve replacement, only 6 (4.1%) patients were found to have amyloid deposits on intraoperative myocardial biopsy. Immunohistochemistry and mass spectroscopy were used to confirm the diagnosis of amyloid subtype and these patients were all found to have wild type transthyretin amyloid by genetic sequencing.12 Once again, a review of routine preprocedural echocardiograms did not raise suspicion for cardiac amyloidosis even in the affected cohort, further emphasizing the difficulty in screening for cardiac amyloidosis using routine methods.
Syncope in patients with transthyretin amyloid cardiomyopathy: clinical features and outcomes
Published in Postgraduate Medicine, 2022
Gonzalo Barge-Caballero, Eduardo Barge-Caballero, Manuel López-Pérez, Raquel Bilbao-Quesada, Eva González-Babarro, Inés Gómez-Otero, Andrea López-López, Mario Gutiérrez-Feijoo, Alfonso Varela-Román, Carlos González-Juanatey, Óscar Díaz-Castro, María G. Crespo-Leiro
Amyloidosis is a family of systemic disorders caused by the abnormal deposition of different types of proteins in organ tissues, including the heart [1,2]. Classically, light-chain amyloid cardiomyopathy associated to hematological disorders has been the most studied and characteristic forms of cardiac amyloidosis [3]. However, nowadays the most frequently diagnosed form of cardiac amyloidosis is the one due to the myocardial deposition of the serum protein transthyretin [4], which itself is subclassified as wild-type transthyretin amyloid cardiomyopathy (ATTR-CM) or variant (hereditary) ATTR-CM [3,5].
Diagnostic and lifetime hospital costs of patients suffering from wild-type transthyretin amyloid cardiomyopathy in Denmark
Published in Journal of Medical Economics, 2020
Trine Pilgaard, Mikkel Hasse Pedersen, Steen Hvitfeldt Poulsen
Wild-type transthyretin amyloid cardiomyopathy (ATTRwt) is a myocardial disease associated with advanced heart failure symptoms and increased morbidity1–6. The epidemiology of ATTRwt is poorly characterized, however, new data suggest that the disease is substantially under- and misdiagnosed5–8. Studies have reported delays in diagnosis from 13 to 78 months9–11. Before the correct diagnosis is established, patients with ATTRwt experience multiple hospitalizations due to misdiagnosis and mistreatments, which has been reported to be as high as six hospital admissions annually9–11.