2017
DOI: 10.1093/eurheartj/ehx043
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Clinical characteristics of wild-type transthyretin cardiac amyloidosis: disproving myths

Abstract: The clinical spectrum of ATTRwt is heterogeneous and differs from the classic phenotype: women are affected in a significant proportion; asymmetric LV hypertrophy and impaired LVEF are not rare and only a minority have low QRS voltages. Clinicians should be aware of the broad clinical spectrum of ATTRwt to correctly identify an entity for which a number of disease-modifying treatments are under investigation.

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Cited by 281 publications
(306 citation statements)
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“…Similar findings, with septal‐to‐posterior wall ratio <1.3, have been reported later by Borer et al In Fabry disease, Linhart et al have shown in the seminal work that concentric LV pattern (either remodeling or hypertrophy) is predominant in FC. Recently, asymmetric septal hypertrophy has been observed in 25% of the patients with wild‐type transthyretin cardiac amyloidosis . Compared to the wild‐type transthyretin amyloid deposition, in our dataset of AL cardiac amyloidosis individuals we have observed a slightly lower percentage (15%); nevertheless, our data show that asymmetric septal hypertrophy can also be present in a minority of AL patients.…”
Section: Discussioncontrasting
confidence: 60%
“…Similar findings, with septal‐to‐posterior wall ratio <1.3, have been reported later by Borer et al In Fabry disease, Linhart et al have shown in the seminal work that concentric LV pattern (either remodeling or hypertrophy) is predominant in FC. Recently, asymmetric septal hypertrophy has been observed in 25% of the patients with wild‐type transthyretin cardiac amyloidosis . Compared to the wild‐type transthyretin amyloid deposition, in our dataset of AL cardiac amyloidosis individuals we have observed a slightly lower percentage (15%); nevertheless, our data show that asymmetric septal hypertrophy can also be present in a minority of AL patients.…”
Section: Discussioncontrasting
confidence: 60%
“…Conduction abnormalities on ECG are also extremely common in cardiac amyloidosis given the infiltrative nature of the disease, though they are not specific and occur commonly with advancing age. Atrial arrhythmias, including atrial flutter and atrial fibrillation, are highly prevalent in ATTRwt cardiac amyloidosis, having a lifetime risk of 90% among patients with ATTRwt cardiac amyloidosis . Heart block and bundle branch block are also very common ECG finding in ATTRwt cardiac amyloidosis and ~30% of subjects require permanent pacing over time.…”
Section: Diagnosismentioning
confidence: 99%
“…Atrial arrhythmias, including atrial flutter and atrial fibrillation, are highly prevalent in ATTRwt cardiac amyloidosis, having a lifetime risk of 90% among patients with ATTRwt cardiac amyloidosis. 28 Heart block and bundle branch block are also very common ECG finding in ATTRwt cardiac amyloidosis and~30% of subjects require permanent pacing over time. A recent study of older patients with conduction disease or HF requiring pacemaker implantation screened patients for ATTR cardiac amyloidosis using nuclear scintigrahy.…”
mentioning
confidence: 99%
“…The association between CTS and systemic amyloidosis has been widely described and the main case series report a high prevalence of CTS in ATTR, ranging from 15% to 60% . Indeed, TTR‐related amyloid deposition has been documented in the transverse carpal ligament of subjects undergoing surgical intervention (with or without associated cardiac involvement) .…”
Section: Introductionmentioning
confidence: 99%