PMID- 25211144 OWN - NLM STAT- MEDLINE DCOM- 20150623 LR - 20220311 IS - 1744-2818 (Electronic) IS - 1350-6129 (Linking) VI - 21 IP - 4 DP - 2014 Dec TI - Noninvasive detection of cardiac involvement in patients with hereditary transthyretin associated amyloidosis using cardiac magnetic resonance imaging: a prospective study. PG - 246-55 LID - 10.3109/13506129.2014.956924 [doi] AB - BACKGROUND: Most of the studies that described cardiac amyloidosis using cardiac magnetic resonance (CMR) imaging refer to patients with primary light chain (AL) amyloidosis. The goal of this study was to evaluate cardiac involvement in patients with hereditary transthyretin associated (ATTR) amyloidosis and asymptomatic carriers and its relationships with clinical symptoms and genotype, using CMR imaging. METHODS AND RESULTS: Fifty-three patients with hereditary ATTR amyloidosis and 14 asymptomatic carriers were included in this study. Morphological, functional and late gadolinium enhancement (LGE) findings were noted on CMR images. A positive LGE suggesting cardiac amyloidosis was detected in 60% of patients. The pattern of LGE was diffuse, focal and circumferential in 32, 26 and 2% of patients, respectively. The inferior basal segment was the most frequently involved (93%) in case of focal involvement. Diffuse pattern was exclusively encountered in patients with cardiac symptoms. Nineteen percent of patients with isolated neurological symptoms and 20% of subjects without left ventricular wall thickening exhibited cardiac abnormalities on CMR. CONCLUSION: Cardiac involvement can be detected in patients with hereditary ATTR amyloidosis with isolated neurological symptoms and without left ventricular wall thickening, suggesting that CMR could be useful in detecting preclinical cardiac amyloidosis. FAU - Deux, Jean-Francois AU - Deux JF AD - Radiology Department, Henri Mondor Hospital, University Paris Est Creteil, Assistance Publique-Hopitaux de Paris , Creteil , France . FAU - Damy, Thibaud AU - Damy T FAU - Rahmouni, Alain AU - Rahmouni A FAU - Mayer, Julie AU - Mayer J FAU - Plante-Bordeneuve, Violaine AU - Plante-Bordeneuve V LA - eng PT - Journal Article DEP - 20140911 PL - England TA - Amyloid JT - Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis JID - 9433802 RN - Amyloidosis, Hereditary, Transthyretin-Related SB - IM MH - Adult MH - Aged MH - Amyloid Neuropathies, Familial/genetics/*physiopathology MH - Female MH - Heart/*physiopathology MH - Humans MH - Magnetic Resonance Imaging/*methods MH - Male MH - Middle Aged MH - Prospective Studies OTO - NOTNLM OT - Amyloid OT - cardiac OT - magnetic resonance imaging OT - transthyretin EDAT- 2014/09/12 06:00 MHDA- 2015/06/24 06:00 CRDT- 2014/09/12 06:00 PHST- 2014/09/12 06:00 [entrez] PHST- 2014/09/12 06:00 [pubmed] PHST- 2015/06/24 06:00 [medline] AID - 10.3109/13506129.2014.956924 [doi] PST - ppublish SO - Amyloid. 2014 Dec;21(4):246-55. doi: 10.3109/13506129.2014.956924. Epub 2014 Sep 11.