Document Detail


MRI of cardiac involvement in transthyretin familial amyloid polyneuropathy.
MedLine Citation:
PMID:  21098170     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVE: The purpose of this study was to evaluate cardiac MRI features in a group of patients with transthyretin familial amyloid polyneuropathy (FAP).
SUBJECTS AND METHODS: Sixteen patients with transthyretin FAP underwent 2D echocardiography with Doppler examination, cardiac MRI, and (99m)Tc-diphosphonate (DPD) scintigraphy. Four patients had peripheral polyneuropathy, three had carpal tunnel syndrome, one patient had symptoms and signs of heart failure, and eight patients had no symptoms but had a family history of FAP. At MRI, cardiac function parameters and delayed contrast enhancement findings were evaluated.
RESULTS: Six patients had cardiac radiotracer uptake at scintigraphy (FAP cardiac group), and 10 patients had no cardiac uptake (FAP noncardiac group). The FAP cardiac group included the four patients with peripheral neuropathy, one patient with carpal tunnel syndrome, and the only patient with heart failure. At MRI, abnormal contrast enhancement was found in all patients with positive scintigraphic findings and in no patient with negative scintigraphic findings. All patients had involvement of the left ventricle and other chambers or structures (atria, right ventricle, tricuspid valve leaflets). Left ventricular contrast enhancement was focal in four patients, subendocardial circumferential in one patient, and diffuse in one patient. The only patient with signs of heart failure had circumferential subendocardial enhancement.
CONCLUSION: Cardiac contrast-enhanced MRI can be used to identify cardiac amyloidosis in patients with FAP who do not have clinical signs of heart involvement. In these patients, the typical subendocardial circumferential pattern of contrast enhancement is rare. We observed unusual enhancement patterns as focal or diffuse left ventricular enhancement accompanied by enhancement of the atria, tricuspid valve, or right ventricle.
Authors:
Gianluca Di Bella; Fabio Minutoli; Anna Mazzeo; Giuseppe Vita; Giuseppe Oreto; Scipione Carerj; Carmelo Anfuso; Massimo Russo; Michele Gaeta
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  AJR. American journal of roentgenology     Volume:  195     ISSN:  1546-3141     ISO Abbreviation:  AJR Am J Roentgenol     Publication Date:  2010 Dec 
Date Detail:
Created Date:  2010-11-24     Completed Date:  2011-01-11     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7708173     Medline TA:  AJR Am J Roentgenol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  W394-9     Citation Subset:  AIM; IM    
Affiliation:
Clinical and Experimental Department of Medicine and Pharmacology, University of Messina, Messina, Italy.
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MeSH Terms
Descriptor/Qualifier:
Adult
Aged
Amyloid Neuropathies, Familial / metabolism*,  pathology*,  radionuclide imaging,  ultrasonography
Contrast Media / diagnostic use
Diphosphonates / diagnostic use
Echocardiography
Female
Humans
Magnetic Resonance Imaging / methods*
Male
Middle Aged
Organometallic Compounds / diagnostic use
Prealbumin / metabolism*
Radiopharmaceuticals / diagnostic use
Technetium Compounds / diagnostic use
Chemical
Reg. No./Substance:
0/Contrast Media; 0/Diphosphonates; 0/Organometallic Compounds; 0/Prealbumin; 0/Radiopharmaceuticals; 0/Technetium Compounds; 138071-82-6/gadobutrol; 52997-55-4/technetium Tc 99m diphosphonate

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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