Atrial Fibrillation in Transthyretin Cardiac Amyloidosis

Donnellan, Eoin; Wazni, Oussama; Hanna, Mazen; El‐Shazly, Mohamed; Puri, Rishi; Saliba, Walid; Kanj, Mohamed; Vakamudi, Sneha; Patel, Divyang; Baranowski, Bryan; Cantillon, Daniel J.; Dresing, Thomas; Jaber, Wael A.

doi:10.1016/j.jacep.2020.04.019

Cited by 104 publications

(88 citation statements)

References 25 publications

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“…Similarly, another retrospective study comprising 360 patients from the Mayo Clinic noted a prevalence of AF and atrial flutter of 62% among individuals with ATTRwt-CA ( 3 ). A further single-center retrospective study from the Cleveland Clinic including 382 patients with ATTR-CA found that AF was present in 76% of patients with ATTRwt-CA and 53% of those with ATTRv-CA ( 19 ).…”

Section: Atrial Fibrillationmentioning

confidence: 99%

“…As previously noted, often times AV nodal conduction disease is present in these patients, allowing for rate-controlled AF without medications. Nevertheless, rate-control agents are sometimes necessary for patients with AF and CA ( 19 , 27 ). Given digoxin’s potential deleterious effects, it was an absolute contraindication in CA patients.…”

Section: Management Of Afmentioning

confidence: 99%

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Electrophysiological Manifestations of Cardiac Amyloidosis

Hartnett¹,

Jaber²,

Maurer³

et al. 2021

JACC: CardioOncology

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Cardiac amyloidosis (CA) is an infiltrative cardiomyopathy caused by the extracellular deposition of amyloid fibrils in the myocardium. Although cardiac amyloidosis patients primarily present with heart failure symptoms, arrhythmias and conduction system disease are frequently encountered. Atrial fibrillation (AF) is observed in up to 70% of patients at the time of diagnosis, and patients typically have controlled ventricular rates caused by concomitant conduction system disease. Thromboembolic risk is particularly high in patients with CA and AF, and left atrial thrombi have been observed even in the absence of clinically diagnosed AF. Atrioventricular nodal and infra-Hisian disease are common, and permanent pacemakers are frequently required. The use of implantable cardioverter-defibrillators in this population is controversial. This review summarizes the published data and therapeutic strategies surrounding arrhythmias and conduction system disease with the goal of aiding clinicians managing the clinical complexities of CA.

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Section: Atrial Fibrillationmentioning

confidence: 99%

Section: Management Of Afmentioning

confidence: 99%

Electrophysiological Manifestations of Cardiac Amyloidosis

Hartnett¹,

Jaber²,

Maurer³

et al. 2021

JACC: CardioOncology

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“…ATTR-CA is associated with a high risk of stroke in itself and independently of conventional risk assessment, such as left atrial size or CHA 2 DS 2 -VASc score [21], and aIAB has been shown to be associated with increased risk of stroke [7,28,29]. Atrial fibrillation is common among patients with ATTR-CA [22], but although atrial fibrillation is a well-established risk factor for stroke, the reported lack of temporal relation suggests that it may not be the direct cause of embolism [30]. Instead, it can be considered (another) risk factor and marker of atrial disease [31].…”

Section: Discussionmentioning

confidence: 99%

“…Secondly, early identification of atrial fibrillation is important in ATTR-CA. Maintained sinus rhythm has been shown to be associated with improved survival, and strategies (both medical and invasive) are more effective in the earlier stages of the disease [22]. Advanced IAB by resting 12-lead ECG is an easily accessible marker for the identification of patients with a high risk of atrial fibrillation and can be used to indicate which patients should undergo further investigations to detect paroxysmal atrial fibrillation, such as long-term ECG recordings.…”

Section: Discussionmentioning

confidence: 99%

“…It is plausible that conduction within the atrium, as well as between the atria, is affected by ATTR-CA, and that the prevalence of aIAB may therefore be increased in this population. Since both stroke and atrial fibrillation are common among patients with ATTR-CA [21,22], it is important to identify reliable predictors of such adverse events.…”

Section: Introductionmentioning

confidence: 99%

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The Prevalence of Advanced Interatrial Block and Its Relationship to Left Atrial Function in Patients with Transthyretin Cardiac Amyloidosis

Lindow

Lindqvist

2021

JCM

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Background: Advanced interatrial block (aIAB), which is associated with incident atrial fibrillation and stroke, occurs in the setting of blocked interatrial conduction. Atrial amyloid deposition could be a possible substrate for reduced interatrial conduction, but the prevalence of aIAB in patients with transthyretin cardiac amyloidosis (ATTR-CA) is unknown. We aimed to describe the prevalence of aIAB and its relationship to left atrial function in patients with ATTR-CA in comparison to patients with HF and left ventricular hypertrophy but no CA. Methods: The presence of aIAB was investigated among 75 patients (49 patients with ATTR-CA and 26 with HF but no CA). A comprehensive echocardiographic investigation was performed in all patients, including left atrial strain and strain rate measurements. Results: Among patients with ATTR-CA, 27% had aIAB and in patients with HF but no CA, this figure was 21%, (p = 0.78). The presence of aIAB was associated with a low strain rate during atrial contraction (<0.91 s−1) (OR: 5.2 (1.4–19.9)), even after adjusting for age and LAVi (OR: 4.5 (1.0–19.19)). Conclusions: Advanced interatrial block is common among patients with ATTR-CA, as well as in patients with heart failure and left ventricular hypertrophy but no CA. aIAB is associated with reduced left atrial contractile function.

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Cardiac Amyloidosis Due to Transthyretin Protein

Ruberg,

Maurer

2024

JAMA

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ImportanceSystemic amyloidosis from transthyretin (ATTR) protein is the most common type of amyloidosis that causes cardiomyopathy.ObservationsTransthyretin (TTR) protein transports thyroxine (thyroid hormone) and retinol (vitamin A) and is synthesized predominantly by the liver. When the TTR protein misfolds, it can form amyloid fibrils that deposit in the heart causing heart failure, heart conduction block, or arrhythmia such as atrial fibrillation. The biological processes by which amyloid fibrils form are incompletely understood but are associated with aging and, in some patients, affected by inherited variants in the TTR genetic sequence. ATTR amyloidosis results from misfolded TTR protein deposition. ATTR can occur in association with normal TTR genetic sequence (wild-type ATTR) or with abnormal TTR genetic sequence (variant ATTR). Wild-type ATTR primarily manifests as cardiomyopathy while ATTR due to a genetic variant manifests as cardiomyopathy and/or polyneuropathy. Approximately 50 000 to 150 000 people in the US have heart failure due to ATTR amyloidosis. Without treatment, heart failure due to ATTR amyloidosis is associated with a median survival of approximately 5 years. More than 130 different inherited genetic variants in TTR exist. The most common genetic variant is Val122Ile (pV142I), an allele with an origin in West African countries, that is present in 3.4% of African American individuals in the US or approximately 1.5 million persons. The diagnosis can be made using serum free light chain assay and immunofixation electrophoresis to exclude light chain amyloidosis combined with cardiac nuclear scintigraphy to detect radiotracer uptake in a pattern consistent with amyloidosis. Loop diuretics, such as furosemide, torsemide, and bumetanide, are the primary treatment for fluid overload and symptomatic relief of patients with ATTR heart failure. An ATTR-directed therapy that inhibited misfolding of the TTR protein (tafamidis, a protein stabilizer), compared with placebo, reduced mortality from 42.9% to 29.5%, reduced hospitalizations from 0.7/year to 0.48/year, and was most effective when administered early in disease course.Conclusions and RelevanceATTR amyloidosis causes cardiomyopathy in up to approximately 150 000 people in the US and tafamidis is the only currently approved therapy. Tafamidis slowed progression of ATTR amyloidosis and improved survival and prevented hospitalization, compared with placebo, in people with ATTR-associated cardiomyopathy.

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Atrial Fibrillation in Transthyretin Cardiac Amyloidosis

Cited by 104 publications

References 25 publications

Electrophysiological Manifestations of Cardiac Amyloidosis

Electrophysiological Manifestations of Cardiac Amyloidosis

The Prevalence of Advanced Interatrial Block and Its Relationship to Left Atrial Function in Patients with Transthyretin Cardiac Amyloidosis

Cardiac Amyloidosis Due to Transthyretin Protein

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