title: Gender-related risk of myocardial involvement in systemic amyloidosis.
abstract: To investigate associations between gender and myocardial involvement in systemic amyloidosis, we reviewed all patients presenting between 1994 and September 2006 in our institutional network (100 AL and 98 familial transthyretin-related amyloidosis (ATTR) patients, plus 12 elderly men with senile systemic amyloidosis). We focused on echocardiographic descriptors of myocardial involvement (height-indexed mean left ventricular (LV) wall thickness, LV mass index), and baseline LV function. Among familial ATTR patients, female prevalence was lower within the highest tertile of either echocardiographic indicator of myocardial involvement. Gender was independently associated with height-indexed mean LV wall thickness (as were gene mutations). Female prevalence appeared rather similar across the different neurological stages. Within the subgroup of familial ATTR patients with amyloidotic cardiomyopathy, women tended to display a considerably less severe morphological and functional echocardiographic profile. We explored the possible role of female sex hormones by considering menopausal status: women in the highest tertile of mean LV wall thickness index were more often postmenopausal than those in the other two tertiles and had a much higher ( approximately 15 years) mean age; analogous age-related associations were not observable for men. In conclusion, these findings raise the hypothesis that some biological characteristic associated with female gender protects against myocardial involvement in familial ATTR.
authors.affiliation: Institute of Cardiology, University of Bologna, Bologna, Italy. claudio.rapezzi@unibo.it
authors.forename: Claudio
authors.initials: C
authors.lastname: Rapezzi
authors.forename: Letizia
authors.initials: L
authors.lastname: Riva
authors.forename: Cristina C
authors.initials: CC
authors.lastname: Quarta
authors.forename: Enrica
authors.initials: E
authors.lastname: Perugini
authors.forename: Fabrizio
authors.initials: F
authors.lastname: Salvi
authors.forename: Simone
authors.initials: S
authors.lastname: Longhi
authors.forename: Paolo
authors.initials: P
authors.lastname: Ciliberti
authors.forename: Francesca
authors.initials: F
authors.lastname: Pastorelli
authors.forename: Elena
authors.initials: E
authors.lastname: Biagini
authors.forename: Ornella
authors.initials: O
authors.lastname: Leone
authors.forename: Robin M T
authors.initials: RM
authors.lastname: Cooke
authors.forename: Letizia
authors.initials: L
authors.lastname: Bacchi-Reggiani
authors.forename: Alessandra
authors.initials: A
authors.lastname: Ferlini
authors.forename: Michele
authors.initials: M
authors.lastname: Cavo
authors.forename: Giampaolo
authors.initials: G
authors.lastname: Merlini
authors.forename: Stefano
authors.initials: S
authors.lastname: Perlini
authors.forename: Sonia
authors.initials: S
authors.lastname: Pasquali
authors.forename: Angelo
authors.initials: A
authors.lastname: Branzi
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D000686:Amyloidosis / Q000150:complications* / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000378:metabolism; D009202:Cardiomyopathies / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000209:etiology* / Q000378:metabolism; D004452:Echocardiography; D005260:Female; D012739:Gonadal Steroid Hormones / Q000378:metabolism; D006352:Heart Ventricles / Q000000981:diagnostic imaging; D006801:Humans; D008297:Male; D008593:Menopause / Q000378:metabolism; D008875:Middle Aged; D012189:Retrospective Studies; D012737:Sex Factors
chemical_list: D012739:Gonadal Steroid Hormones