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Amyloid Cardiomyopathy in Hereditary Transthyretin V30M Amyloidosis - Impact of Sex and Amyloid Fibril Composition
Umea Univ, Dept Clin Physiol, Ctr Heart, Umea, Sweden.;Umea Univ, Dept Publ Hlth & Clin Med, Umea, Sweden..
Umea Univ, Dept Cardiol, Ctr Heart, Umea, Sweden.;Umea Univ, Dept Publ Hlth & Clin Med, Umea, Sweden..
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology.
Umea Univ, Dept Clin Physiol, Ctr Heart, Umea, Sweden.;Umea Univ, Dept Surg & Perioperat Sci, Umea, Sweden..
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2015 (English)In: PLoS ONE, ISSN 1932-6203, E-ISSN 1932-6203, Vol. 10, no 11, e0143456Article in journal (Refereed) Published
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Abstract [en]

Purpose Transthyretin V30M (ATTR V30M) amyloidosis is a phenotypically diverse disease with symptoms ranging from predominant neuropathy to exclusive cardiac manifestations. The aims of this study were to determine the dispersion of the two types of fibrils found in Swedish ATTR V30M patients - Type A consisting of a mixture of truncated and full length ATTR fibrils and type B fibrils consisting of full length fibrils, and to estimate the severity of cardiac dysfunction in relation to fibril composition and sex. Material and Methods Echocardiographic data were analysed in 107 Swedish ATTR V30M patients with their fibril composition determined as either type A or type B. Measurements of left ventricular (LV) dimensions and evaluation of systolic and diastolic function including speckle tracking derived strain were performed. Patients were grouped according to fibril type and sex. Multivariate linear regression was utilised to determine factors of significant impact on LV thickness. Results There was no significant difference in proportions of the two types of fibrils between men and women. In patients with type A fibrils, women had significantly lower median septal (p = 0.007) and posterior wall thicknesses (p = 0.010), lower median LV mass indexed to height (p = 0.008), and higher septal strain (p = 0.037), as compared to males. These differences were not apparent in patients with type B fibrils. Multiple linear regression analysis revealed that fibril type, sex and age all had significant impact on LV septal thickness. Conclusion This study demonstrates a clear difference between sexes in the severity of amyloid heart disease in ATTR V30M amyloidosis patients. Even though type A fibrils were associated with more advanced amyloid heart disease compared to type B, women with type A fibrils generally developed less cardiac infiltration than men. The differences may explain the better outcome for liver transplanted late-onset female patients compared to males.

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2015. Vol. 10, no 11, e0143456
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Medical and Health Sciences
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URN: urn:nbn:se:uu:diva-272284DOI: 10.1371/journal.pone.0143456ISI: 000365853900127OAI: oai:DiVA.org:uu-272284DiVA: diva2:894279
Funder
Swedish Heart Lung Foundation
Available from: 2016-01-14 Created: 2016-01-13 Last updated: 2017-11-30Bibliographically approved

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Westermark, Per

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