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Heart complications in familial transthyretin amyloidosis: impact of age and gender.
Umeå University, Faculty of Medicine, Department of Surgical and Perioperative Sciences, Clinical Physiology.
Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
Umeå University, Faculty of Medicine, Department of Radiation Sciences, Radiation Physics.
Umeå University, Faculty of Medicine, Department of Surgical and Perioperative Sciences, Clinical Physiology.
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2010 (English)In: Amyloid: Journal of Protein Folding Disorders, ISSN 1350-6129, E-ISSN 1744-2818, Vol. 17, no 2, 63-68 p.Article in journal (Refereed) Published
Abstract [en]

Heart arrhythmia is common in Swedish patients with familial amyloidotic polyneuropathy (FAP), as well as cardiomyopathy. We investigated the relationship between Holter ECG and echocardiographic findings in 108 FAP patients, with particular focus on age and gender differences. Female patients were younger than male patients at symptom onset (p < 0.01). Only 4 of 39 patients with septal hypertrophy were females. Regression analysis showed that age of onset, gender and duration of disease were significantly related with intraventricular septum (IVS) thickness. Sixty-five patients (25 females) presented with abnormal 24-h ECG recordings. IVS thickness was not significantly related to conduction disturbances or the presence of ventricular arrhythmia (VA). However, IVS thickness and atrial dimension were both related to increased rate of supraventricular arrhythmia (SVA). Male gender was clearly associated with more pronounced septal thickness of the heart. Conduction disturbances were not related to IVS thickness, indicating that the distribution and extent of infiltration of the heart by amyloid are heterogeneous and related to gender and age of onset. These findings highlight the necessity of 24-h ECGs to detect conduction disturbances, due to their occurrence in the absence of echocardiographic evidence of amyloid deposition in the myocardium.

Place, publisher, year, edition, pages
2010. Vol. 17, no 2, 63-68 p.
Keyword [en]
Arrhythmia, echocardiography, FAP, Holter
National Category
Medical and Health Sciences
Identifiers
URN: urn:nbn:se:umu:diva-34885DOI: 10.3109/13506129.2010.483114ISI: 000279801200003PubMedID: 20462364OAI: oai:DiVA.org:umu-34885DiVA: diva2:326567
Available from: 2010-06-23 Created: 2010-06-23 Last updated: 2017-12-12Bibliographically approved

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Hörnsten, RolfWiklund, UrbanLindqvist, PerJensen, Steen MSuhr, Ole B

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