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Reduced left atrial myocardial deformation irrespective of cavity size: a potential cause for atrial arrhythmia in hereditary transthyretin amyloidosis
Umea Univ, Publ Hlth & Clin Med, Umea, Sweden.
Umea Univ, Publ Hlth & Clin Med, Umea, Sweden.
Umea Univ, Ctr Heart, Clin Physiol, Umea, Sweden.
Umea Univ, Publ Hlth & Clin Med, Umea, Sweden.
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2018 (English)In: Amyloid: Journal of Protein Folding Disorders, ISSN 1350-6129, E-ISSN 1744-2818, Vol. 25, no 1, p. 46-53Article in journal (Refereed) Published
Abstract [en]

Background: Cardiac amyloidosis (CA) is a myocardial disease and commonly under-diagnosed condition. In CA patients, atrial fibrillation might occur in the absence of left atrial (LA) enlargement.

Objectives: The aim of this study is to assess LA size and function, and its relationship with atrial arrhythmia in patients with hereditary transthyretin amyloidosis (ATTR).

Methods: Forty-six patients with confirmed ATTR amyloidosis on abdominal biopsy were studied. Assessment with 2D echocardiography and 2D strain showed 31 patients had increased LV wall thickness (LVWT) (septal thickness >12mm), and 15 had normal LVWT. In addition to conventional measurements, LV and LA global longitudinal strain (GLS%) and strain rate (SR) were obtained. Western blot analysis was done to assess fibril type. ATTR patients with increased LVWT were compared with 23 patients with hypertrophic cardiomyopathy (HCM) and 31 healthy controls. ATTR amyloidosis patients also underwent 24hour Holter monitoring to determine the presence of atrial arrhythmia.

Results: Atrial deformation during atrial systole was reduced in ATTR amyloidosis patients with increased LVWT independent of LA size and in contrast to HCM. Twenty of the ATTR amyloidosis patients (54%) had ECG evidence of significant atrial arrhythmic events. LA strain rate, during atrial systole, was the only independent predictor of atrial arrhythmia (=3.28, p=.012).

Conclusion: In ATTR cardiomyopathy with increased LVWT, LA myocardial function is abnormal, irrespective of atrial cavity size. Reduced LA myocardial SR during atrial systole, irrespective of cavity volume, E/e and LV deformation, is also a strong predictor for atrial arrhythmic events.

Place, publisher, year, edition, pages
TAYLOR & FRANCIS LTD , 2018. Vol. 25, no 1, p. 46-53
Keywords [en]
Arrhythmia, left atrial function, deformation echocardiography, Holter ECG
National Category
Cardiac and Cardiovascular Systems Clinical Laboratory Medicine
Research subject
Pathology
Identifiers
URN: urn:nbn:se:uu:diva-354533DOI: 10.1080/13506129.2018.1430027ISI: 000428570300007PubMedID: 29369708OAI: oai:DiVA.org:uu-354533DiVA, id: diva2:1234201
Funder
Swedish Heart Lung FoundationErik, Karin och Gösta Selanders FoundationAvailable from: 2018-07-23 Created: 2018-07-23 Last updated: 2019-03-29Bibliographically approved

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