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99mTC-DPD uptake reflects amyloid fibril composition in hereditary transthyretin amyloidosis
Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Cardiology.
Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
Department of Immunology, Genetics and Pathology, Uppsala University, Uppsala, Sweden.
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2016 (English)In: Upsala Journal of Medical Sciences, ISSN 0300-9734, E-ISSN 2000-1967, Vol. 121, no 1, 17-24 p.Article in journal (Refereed) PublishedText
Abstract [en]

Aims In transthyretin amyloid (ATTR) amyloidosis various principal phenotypes have been described: cardiac, neuropathic, or a mixed cardiac and neuropathic. In addition, two different types of amyloid fibrils have been identified (type A and type B). Type B fibrils have thus far only been found in predominantly early-onset V30M and in patients carrying the Y114C mutation, whereas type A is noted in all other mutations currently examined as well as in wild-type ATTR amyloidosis. The fibril type is a determinant of the ATTR V30M disease phenotype. Tc-99m-DPD scintigraphy is a highly sensitive method for diagnosing heart involvement in ATTR amyloidosis. The objective of this study was to determine the relationship between ATTR fibril composition and Tc-99m-DPD scintigraphy outcome in patients with biopsy-proven ATTR amyloidosis. Methods Altogether 55 patients with biopsy-proven diagnosis of ATTR amyloidosis and amyloid fibril composition determined were examined by Tc-99m-DPD scintigraphy. The patients were grouped and compared according to their type of amyloid fibrils. Cardiovascular evaluation included ECG, echocardiography, and cardiac biomarkers. The medical records were scrutinized to identify subjects with hypertension or other diseases that have an impact on cardiac dimensions. Results A total of 97% with type A and none of the patients with type B fibrils displayed Tc-99m-DPD uptake at scintigraphy (p < 0.001). Findings from analyses of cardiac biomarkers, ECG, and echocardiography, though significantly different, could not differentiate between type A and B fibrils in individual patients. Conclusion In ATTR amyloidosis, the outcome of Tc-99m-DPD scintigraphy is strongly related to the patients' transthyretin amyloid fibril composition.

Place, publisher, year, edition, pages
Taylor & Francis, 2016. Vol. 121, no 1, 17-24 p.
Keyword [en]
transthyretin, Amyloidosis hereditary, echocardiography, scintigraphy, amyloid cardiomyopathy, VEREUX RB, 1986, AMERICAN JOURNAL OF CARDIOLOGY, V57, P450 bue G, 2003, AMYLOID-JOURNAL OF PROTEIN FOLDING DISORDERS5th Int Symp on Familial yloidotic Polyneuropathy and Other Transthyretin Related Disorders/4th Int Workshop on Liver ansplantation in Familial Amyloid Polyneuropathy, SEP 24-27, 2002, MATSUMOTO, JAPAN, V10, P32 gueh Sherif F., 2009, EUROPEAN JOURNAL OF ECHOCARDIOGRAPHY, V10, P165 ille M, 2002, EUROPEAN JOURNAL OF NUCLEAR MEDICINE AND MOLECULAR IMAGING, V29, P376
National Category
Clinical Medicine
URN: urn:nbn:se:umu:diva-119085DOI: 10.3109/03009734.2015.1122687ISI: 000372123700003PubMedID: 26849806OAI: diva2:920952
Available from: 2016-04-19 Created: 2016-04-11 Last updated: 2016-04-19Bibliographically approved

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Suhr, Ole B.Näslund, UlfLindqvist, PerSundström, Torbjörn
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