Document Detail

The electrocardiographic features associated with cardiac amyloidosis of variant transthyretin isoleucine 122 type in Afro-Caribbean patients.
MedLine Citation:
PMID:  22795285     Owner:  NLM     Status:  In-Data-Review    
BACKGROUND: About 4% of African Americans possess the isoleucine 122 (V122I) variant of transthyretin, associated with cardiac amyloidosis beyond ages of 55 to 60 years. Transthyretin amyloidosis associated with variant V122I (ATTR V122I) is likely to be an important cause of heart failure in Afro-Caribbean populations, but the high prevalence of left ventricular hypertrophy (LVH) and lack of awareness of this genetic disorder pose diagnostic hurdles. We report the electrocardiographic (ECG) features of ATTR V122I in the largest clinical series to date.
METHODS: Patients with ATTR V122I were identified in collaboration with the UK National Amyloidosis Centre. The ECG at presentation was assessed for cardiac rhythm, axis, and voltage complex size.
RESULTS: We include 64 patients with ATTR V122I, with a median age of 74 years (range, 57-88 years). Normal or increased ECG voltage was present in 44.3% of patients, and overall 25% met the criteria for LVH. A significant negative correlation between voltage complex size and duration of illness was seen (P < .05). First-degree heart block was evident in 56% of patients in sinus rhythm. During follow-up (n = 17; median, 28 months), 50% of patients with initial first-degree heart block required pacing.
CONCLUSION: Electrocardiographic voltages meet the criteria for LVH in one quarter of patients with ATTR V122I cardiac amyloidosis. The widely held belief that cardiac amyloidosis is associated with low-voltage complexes is likely to contribute to underdiagnosis of ATTR V122I. First-degree heart block is common at diagnosis and identifies patients at high risk for subsequent pacing requirement.
Jason Dungu; Prayman T Sattianayagam; Carol J Whelan; Simon D J Gibbs; Jennifer H Pinney; Sanjay M Banypersad; Dorota Rowczenio; Janet A Gilbertson; Helen J Lachmann; Ashutosh Wechalekar; Julian D Gillmore; Philip N Hawkins; Lisa J Anderson
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  American heart journal     Volume:  164     ISSN:  1097-6744     ISO Abbreviation:  Am. Heart J.     Publication Date:  2012 Jul 
Date Detail:
Created Date:  2012-07-16     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0370465     Medline TA:  Am Heart J     Country:  United States    
Other Details:
Languages:  eng     Pagination:  72-9     Citation Subset:  AIM; IM    
Copyright Information:
Copyright © 2012 Mosby, Inc. All rights reserved.
National Amyloidosis Centre, UCL Medical School, Royal Free Campus, London, United Kingdom; St George's University of London, London, United Kingdom.
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