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Journal articles on the topic 'ATTR amyloidosys, Arrhythmia'

Author: Grafiati

Published: 10 March 2023

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1

Donnellan, Eoin, Oussama Wazni, Mohamed Kanj, et al. "Atrial fibrillation ablation in patients with transthyretin cardiac amyloidosis." EP Europace 22, no. 2 (2019): 259–64. http://dx.doi.org/10.1093/europace/euz314.

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Abstract Aims Atrial fibrillation (AF) occurs in as many as 70% of patients with transthyretin cardiac amyloidosis (ATTR CA). The aim of our study was to investigate the impact of AF ablation on freedom from recurrent arrhythmia, hospitalization for AF or heart failure (HF), and mortality. Methods and results This was a retrospective observational cohort study of 72 patients with ATTR CA and AF, of whom 24 underwent AF ablation and were matched in a 2:1 manner based on age, gender, ATTR CA stage, New York Heart Association functional class, ejection fraction, and date of AF diagnosis with 48 p

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2

Nagarakanti, Rangadham, Kesavan Sankaramangalam, Sindhu Nagarakanti, Danyaal Moin, Kenneth Dulnuan, and Deepa B. Iyer. "Cardiac Amyloidosis Wild Type (ATTR-CAwt) and Associated Cardiac Arrhythmias: A Case Report and Literature Review." Indian Journal of Clinical Cardiology 2, no. 2 (2021): 80–85. http://dx.doi.org/10.1177/2632463621998583.

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Cardiac amyloidosis wild type (ATTR-CAwt) is often the underdiagnosed cause of heart failure with preserved ejection fraction (HFpEF) and has a high mortality rate. There is usually a long delay between the appearance of clinical signs and the diagnosis of ATTR-CAwt but a short duration between diagnosis and death. ATTR-CAwt was associated with significant clinical arrhythmias. We report a case of ATTR-CAwt, the process of its diagnosis, and the associated clinical arrhythmias and their management. We also reviewed the literature of this underdiagnosed and potentially fatal condition and the c

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3

Khayambashi, S., C. Hahn, N. Fine, E. Mahe, and K. Elzinga. "P.045 Biopsies of the transverse carpal ligament and tenosynovium for tissue confirmation of transthyretin amyloidosis." Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques 49, s1 (2022): S19. http://dx.doi.org/10.1017/cjn.2022.146.

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Background: Transthyretin Amyloidosis (ATTR) is a common cause of both cardiomyopathy and carpal tunnel syndrome, with many patients needing carpal tunnel release (CTR). Although tafamidis is now an approved treatment of ATTR cardiomyopathy, insurers in most provinces require biopsy confirmation of amyloidosis. Endomyocardial biopsy is often the chosen approach due to optimal sensitivity, albeit with risk of serious adverse events such as stroke, cardiac tamponade, and arrhythmias. CTR may present an ideal opportunity for obtaining amyloidosis biopsy confirmation. Methods: ATTR patients underg

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4

Aouate, David, Aymeric Menet, Dimitri Bellevre, Thibaud Damy, and Sylvestre Marechaux. "Deleterious effect of right ventricular pacing in patients with cardiac transthyretin amyloidosis: potential clinical benefit of cardiac resynchronization therapy." European Heart Journal - Case Reports 4, no. 3 (2020): 1–5. http://dx.doi.org/10.1093/ehjcr/ytaa088.

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Abstract Background Cardiac amyloidosis involvement is associated with a detrimental outcome including frequent arrhythmias, heart failure, and conduction disturbances which may need permanent pacing. Cases summary We report two cases of patients with transthyretin amyloidosis (ATTR) who developed heart failure and depressed left ventricular ejection fraction (LVEF) following permanent right ventricular (RV) pacing but highly responded to cardiac resynchronization therapy (CRT). Discussion The impact of RV pacing and CRT in cardiac amyloidosis is not known. In our cases, the detrimental effect

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5

Juarez, Michel, Gaspar Del Rio-Pertuz, Kanak Parmar, Melanie C. Bois, Scott Shurmur, and Erwin Argueta-Sosa. "A Case of Early Hereditary Transthyretin Amyloid Cardiomyopathy Recognition With Genetic Screening: A Case Report." Journal of Primary Care & Community Health 13 (January 2022): 215013192110626. http://dx.doi.org/10.1177/21501319211062682.

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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 ag

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6

Kim, Eun-Jeong, Benjamin B. Holmes, Shi Huang, et al. "Outcomes in patients with cardiac amyloidosis and implantable cardioverter-defibrillator." EP Europace 22, no. 8 (2020): 1216–23. http://dx.doi.org/10.1093/europace/euaa094.

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Abstract Aims Cardiac amyloidosis (CA) is associated with increased mortality due to arrhythmias, heart failure, and electromechanical dissociation. However, the role of an implantable cardioverter-defibrillator (ICD) remains unclear. We conducted case-control study to assess survival in CA patients with and without a primary prevention ICD and compared outcomes to an age, sex, and device implant year-matched non-CA group with primary prevention ICD. Methods and results There were 91 subjects with CA [mean age= 71.2 ± 10.2, female 22.0%, 49 AL with Mayo Stage 2.9 ± 1.0, 41 transthyretin amyloi

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7

Antonelli, Giorgio, Valeria Cammalleri, Valeria Maria De Luca, et al. "957 EMERGING FROM THE DARKNESS. SUDDEN CARDIAC DEATH IN CARDIAC AMYLOIDOSIS." European Heart Journal Supplements 24, Supplement_K (2022). http://dx.doi.org/10.1093/eurheartjsupp/suac121.581.

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Abstract Cardiac amyloidosis (CA) manifests as infiltrative cardiomyopathy with a hypertrophic pattern, usually presenting with heart failure with preserved ejection fraction (HFpEF). In addition, degenerative valvular heart disease, particularly severe aortic stenosis, is commonly seen in patients with CA. However, amyloid fibril deposition may also infiltrate the conduction system and promote the development of electrical disorders, including ventricular tachyarrhythmias (VT), atrio-ventricular block, or acute electromechanical dissociation (EMD). These manifestations can increase the risk o

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8

Torselletti, Lorenzo, Stronati Giulia, Alessandro Barbarossa, et al. "178 ARRHYTHMIC STORM IN ATTR WILD TYPE AMYLOIDOSIS: AN UNUSUAL COMBINATION." European Heart Journal Supplements 24, Supplement_K (2022). http://dx.doi.org/10.1093/eurheartjsupp/suac121.087.

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Abstract Cardiac amyloidosis (CA) is characterized by extracellular protein fibril deposition in the myocardium leading to restrictive heart failure. Both atrial and ventricular arrhythmias are common in CA. Study have shown up to one half of patients with CA die suddenly. However, the most common cause of sudden death has been historically through to be secondary electromechanical dissociation rather than a lethal ventricular arrhythmia. We present the case of a 84 years old man, with a history of hypertension, dyslipidaemia, and prior smoking. In June 2020 the patient was admitted to the eme

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9

D'Agostino, S., G. Gizzi, and V. Parato. "P289 EYESHOT OF CARDIAC AMYLOIDOSIS CASES PRESENTED TO A CARDIOLOGY UO IN CENTRAL ITALY IN 2021." European Heart Journal Supplements 24, Supplement_C (2022). http://dx.doi.org/10.1093/eurheartj/suac012.278.

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Abstract Introduction ATTR–related cardiac amyloidosis, caused by the accumulation of insoluble fibrillar proteins derived from transthyretin misfolding, is an often underdiagnosed pathology, especially when neurological involvement is absent or minor. Transthoracic echocardiography represents the first fundamental step in the diagnostic process. Abstract During the year 2021, a total of 9 patients with an echocardiographic feature of hypertrophic heart disease suggestive of infiltrative pathology were referred to our Cardiology unit. Of these patients, 89% were male (8 out of 9), aged between

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10

Cho, Jae Hyung, Natasha Cuk, Derek Leong, et al. "Abstract 16235: Rhythmic Disturbances in Patients With Cardiac Amyloidosis: Analysis From Cedars-Sinai Amyloidosis Registry." Circulation 142, Suppl_3 (2020). http://dx.doi.org/10.1161/circ.142.suppl_3.16235.

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Introduction: Cardiac arrhythmias are frequent complications of patients with cardiac amyloidosis. The burden and nature of cardiac arrhythmias in patients with cardiac amyloidosis have not been investigated in a large patient population. Hypothesis: Cardiac amyloidosis is a highly arrhythmogenic disorder requiring implantation of cardiac electronic devices. Methods: Cedars-Sinai amyloidosis registry was analyzed to investigate the prevalence and nature of cardiac arrhythmias in patients with cardiac amyloidosis. Ambulatory ECG monitoring data, implantable cardioverter-defibrillator or pacemak

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11

Zhang, K. W., R. Zhang, Y. Soyama, M. Karmpalioti, D. J. Lenihan, and J. Gorcsan. "P2724Diagnosis of transthyretin versus light chain cardiac amyloidosis by apical sparing strain ratio in patients with clinically suspected disease." European Heart Journal 40, Supplement_1 (2019). http://dx.doi.org/10.1093/eurheartj/ehz748.1041.

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Abstract Background Apical sparing by longitudinal strain imaging has reported utility for the diagnosis of cardiac amyloidosis. However, potential differences in the apical sparing pattern in light chain (AL) versus transthyretin (ATTR) amyloidosis in patients with high clinical suspicion for cardiac amyloidosis is not clear. Purpose Our objective was to test the hypothesis that echocardiographic strain imaging could determine differences in patients with clinically suspected AL and ATTR cardiac amyloidosis. Methods We studied 206 patients, aged 64±11, with clinically suspected cardiac amyloi

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12

Fede, Giuseppe, Giuseppe Abate, Paolo Belluardo, et al. "515 Exertional syncope and ventricular arrhythmia in a patient with cardiac amyloidosis." European Heart Journal Supplements 23, Supplement_G (2021). http://dx.doi.org/10.1093/eurheartj/suab141.002.

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Abstract Aims Systemic amyloidosis is a multisystem disorder caused by the misfolding, aggregation, and deposition of certain proteins in various organs and tissues. Cardiac involvement is common and worsens the prognosis. Atrial fibrillation is the most frequent arrhythmia in cardiac amiloidosis (CA), but many cases of ventricular arrhythmias (VA) and sudden cardiac death (SCD) have been described. Methods and results We present a case of a 64-year-old man admitted following exertional syncope occurred after climbing a flight of stairs. In the previous 2 weeks he had experienced fatigue and p

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13

Irabor, Biobelemoye, Jacqueline M. McMillan, and Nowell M. Fine. "Assessment and Management of Older Patients With Transthyretin Amyloidosis Cardiomyopathy: Geriatric Cardiology, Frailty Assessment and Beyond." Frontiers in Cardiovascular Medicine 9 (May 17, 2022). http://dx.doi.org/10.3389/fcvm.2022.863179.

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Transthyretin amyloidosis cardiomyopathy (ATTR-CM) is commonly diagnosed in older adults, in particular the wild-type (ATTRwt), which is regarded as an age-related disease. With an aging population and improved diagnostic techniques, the prevalence and incidence of ATTR-CM will continue to increase. With increased availability of mortality reducing ATTR-CM therapies, patients are living longer. The predominant clinical manifestation of ATTR-CM is heart failure, while other cardiovascular manifestations include arrhythmia and aortic stenosis. Given their older age at diagnosis, patients often p

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14

Lamb, Nick. "Why the Diagnosis of ATTR-Cardiomyopathy May Be a Challenge for Physicians." EMJ Cardiology, October 17, 2019, 66–71. http://dx.doi.org/10.33590/emjcardiol/10310030.

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Cardiac amyloidosis is a rare but life-threatening group of disorders caused by the extracellular deposition of misfolded amyloid fibrils in cardiac tissue. Amyloid accumulation leads to cardiomyocyte toxicity, extracellular volume expansion, and ventricular pseudohypertrophy. Two types of amyloid protein are thought to be responsible for most disorders: immunoglobulin light chain, which causes light chain amyloidosis (AL); and transthyretin (TTR), which causes transthyretin amyloidosis (ATTR), of which there are two types: hereditary (hATTR) or wild-type (ATTRwt). Despite increasing clinical

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15

Rahman, Tanvir, Reihaneh C. Moghadam, Vikram V. Agarwal, and Craig K. Reiss. "Cardiac amyloidosis: diagnostic challenges and recent advancement in the treatment of transthyretin amyloidosis (ATTR)." Oxford Medical Case Reports 2021, no. 8 (2021). http://dx.doi.org/10.1093/omcr/omab059.

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ABSTRACT ATTR-CA is an under-reported cause of congestive heart failure (CHF) and cardiac arrhythmias. Heightened clinical suspicion along with a multimodal investigative approach is often required in diagnosing this potentially fatal condition. Tafamidis and inotersen have shown promising results in terms of progression-free survival by ameliorating CHF symptoms and peripheral neuropathies in clinical trials. In this case series of five patients, we present three wild-type cardiac amyloidosis (ATTRwt-CA), one familial cardiac amyloidosis (ATTRm-CA) and one primary cardiac (AL-CA). The diagnos

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16

Guerra, F., F. Coretti, L. Torselletti, et al. "Prognostic role of low QRS voltages in patients with cardiac amyloidosis." European Heart Journal 43, Supplement_2 (2022). http://dx.doi.org/10.1093/eurheartj/ehac544.1781.

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Abstract Introduction Cardiac amyloidosis (CA) is an underdiagnosed and heterogeneous cardiac disease characterized by the extracellular deposition of misfolded proteins in the cardiac tissue. Clinical manifestations are heterogeneous leading to progressive heart failure, often complicated by arrhythmias and conduction system disease. Among several sign and symptoms that are suspicious for the disease, named “red flags”, disproportionally low QRS voltages on the ECG has been described. Purpose The aim of this prospective observational study is to evaluate potential prognostic features of QRS a

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17

Dale, Zack, Lana Rashdan, Pranav Chandrashekar, Stephen B. Heitner, Babak Nazer, and Ahmad Masri. "Abstract 16465: Mode of Death and Outcomes of Implantable Cardioverter Defibrillators in Transthyretin Cardiac Amyloidosis." Circulation 142, Suppl_3 (2020). http://dx.doi.org/10.1161/circ.142.suppl_3.16465.

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Introduction: Patients with transthyretin cardiac amyloidosis (ATTR-CM) may have higher risk of sudden cardiac death yet the role of implantable cardioverter defibrillators (ICD) remains poorly defined. We report our experience with ICDs in a cohort with ATTR-CM. Methods: A single center observational cohort study of patients who had been seen at our Amyloidosis Center with either wild-type or hereditary ATTR-CM diagnosed between 2005-2019. ICD implant was at discretion of treating cardiologists. Device data was obtained from record review. Results: Ninety-seven patients with ATTR-CM (mean age

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18

Azevedo Coutinho, M. D. C., N. Cortez-Dias, G. Cantinho, et al. "Diagnostic and prognostic contribution of 99mTc-DPD scintigraphy in transthyretin V30M cardiac amyloidosis." European Heart Journal 42, Supplement_1 (2021). http://dx.doi.org/10.1093/eurheartj/ehab724.0278.

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Abstract Background Early diagnosis and prognostic stratification of hereditary transthyretin amyloidosis (ATTR) are crucial. Previous findings suggested that 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid (DPD) scintigraphy presents suboptimal accuracy to detect ATTR cardiomyopathy caused by V30M mutation, particularly in patients with onset of symptoms under 50 years of age. Furthermore, its prognostic value has never been evaluated in ATTR caused by this mutation. Purpose To assess the diagnostic value of DPD scintigraphy to detect cardiomyopathy in a large cohort of patients with ATTR-

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19

Damy, T., G. Bourel, M. Slama, et al. "Epidemiology of transthyretin amyloid cardiomyopathy (ATTR-CM) in France, a study based on the systeme national des donnees de sante (SNDS) the French nationwide claims database." European Heart Journal 42, Supplement_1 (2021). http://dx.doi.org/10.1093/eurheartj/ehab724.3131.

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Abstract Transthyretin (TTR) Amyloid Cardiomyopathy (ATTR-CM) is a rare, progressively debilitating, fatal disease with poor prognosis caused by amyloid deposition of fibrils derived from the serum protein TTR in the extracellular matrix of the heart. As amyloid infiltration in the heart progresses, atrial and ventricular walls thicken and become restrictive, resulting in diastolic dysfunction and further progression leads to heart failure, usually with preserved ejection fraction. Systolic dysfunction occurs in the late stages of the disease. Cardiac symptoms and conduction abnormalities also

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20

Dale, Z., P. Chandrashekar, L. Al-Rashdan, M. Kim, A. Masri, and B. Nazer. "Management strategies for atrial fibrillation and flutter in patients with transthyretin cardiac amyloidosis." European Heart Journal 42, Supplement_1 (2021). http://dx.doi.org/10.1093/eurheartj/ehab724.1810.

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Abstract Background Atrial fibrillation (AF) and flutter (AFL) are common in transthyretin cardiac amyloidosis (ATTR-CM). Ventricular rate control drugs in ATTR-CM are poorly tolerated but data addressing tolerability and efficacy of rhythm control strategies is limited. Purpose We report characteristics and outcomes of AF/AFL in a cohort with ATTR-CM. Methods A single center observational study of patients seen at our Amyloidosis Center with wild-type or hereditary ATTR-CM diagnosed between 2005–2019. Treatment was prescribed as per treating cardiologists. Results Eighty-four patients with AT

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21

Quarta, C. Cristina, Marianna Fontana, Thibaud Damy, et al. "Changing paradigm in the treatment of amyloidosis: From disease-modifying drugs to anti-fibril therapy." Frontiers in Cardiovascular Medicine 9 (December 20, 2022). http://dx.doi.org/10.3389/fcvm.2022.1073503.

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Cardiac amyloidosis is a rare, debilitating, and usually fatal disease increasingly recognized in clinical practice despite patients presenting with non-specific symptoms of cardiomyopathy. The current standard of care (SoC) focuses on preventing further amyloid formation and deposition, either with anti-plasma cell dyscrasia (anti-PCD) therapies in light-chain (AL) amyloidosis or stabilizers of transthyretin (TTR) in transthyretin amyloidosis (ATTR). The SoC is supplemented by therapies to treat the complications arising from organ dysfunction; for example, heart failure, arrhythmia, and prot

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22

De Michieli, Laura, Giulio Sinigiani, Petra Deola, et al. "169 CARDIAC AMYLOIDOSIS AND CORONARY ARTERY DISEASE: INSIGHTS FROM A RETROSPECTIVE OBSERVATIONAL STUDY." European Heart Journal Supplements 24, Supplement_K (2022). http://dx.doi.org/10.1093/eurheartjsupp/suac121.606.

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Abstract Introduction Patients with cardiac amyloidosis (CA) frequently present with heart failure with various extra-cardiac red flags. However, some patients can present with typical or atypical chest pain or even mimicking an acute coronary syndrome. Significant epicardial coronary artery disease (CAD) and CA can coexist, but microvascular dysfunction might also play a role. The aims of this study were to systematically investigate clinical indications for invasive coronary angiography (ICA) in patients with CA, to verify the prevalence of significant epicardial CAD and its relation to the

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23

Funabashi, N., and Y. Kobayashi. "Prediction of sites of wall thickening and abnormal late enhancement on cardiac CT and magnetic resonance imaging using electrocardiography findings in patients with confirmed cardiac amyloidosis." European Heart Journal 42, Supplement_1 (2021). http://dx.doi.org/10.1093/eurheartj/ehab724.0309.

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Abstract Background Left ventricular (LV) wall thickening and diastolic dysfunction on a transthoracic echocardiogram (TTE) without high voltage R wave in V5 leads on ECG leads to a diagnosis of cardiac amyloidosis. A final diagnosis is made by endomyocardial biopsy. However, amyloid sometimes invade the right ventricle (RV), and left (LA) and right (RA) atria to cause ECG changes such as sick sinus syndrome (SSS), arrhythmia, and QRS wave axis deviation. Purpose To predict sites of wall thickening and abnormal late enhancement (LE) on cardiac computed tomography (CT) and magnetic resonance im

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24

Funabashi, N., and Y. Kobayashi. "Comparison of sites of wall thickening and abnormal late enhancement on cardiac CT and magnetic resonance imaging with electrocardiography findings in patients with confirmed cardiac amyloidosis." European Heart Journal 43, Supplement_1 (2022). http://dx.doi.org/10.1093/eurheartj/ehab849.010.

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Abstract Funding Acknowledgements Type of funding sources: None. Background Left ventricular (LV) wall thickening and diastolic dysfunction on a transthoracic echocardiogram (TTE) without a high voltage R wave on V5 leads on an ECG leads to a diagnosis of cardiac amyloidosis. A final diagnosis is made by endomyocardial biopsy. However, amyloid sometimes invades the right ventricle (RV), and left (LA) and right (RA) atria, causing ECG changes such as sick sinus syndrome (SSS), arrhythmia, and QRS wave axis deviation. Purpose To elucidate the relationship between sites of wall thickening and abn

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