Gender-related risk of myocardial involvement in systemic amyloidosis.
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.
Rapezzi
Claudio
C
Institute of Cardiology, University of Bologna, Bologna, Italy. claudio.rapezzi@unibo.it
Riva
Letizia
L
Quarta
Cristina C
CC
Perugini
Enrica
E
Salvi
Fabrizio
F
Longhi
Simone
S
Ciliberti
Paolo
P
Pastorelli
Francesca
F
Biagini
Elena
E
Leone
Ornella
O
Cooke
Robin M T
RM
Bacchi-Reggiani
Letizia
L
Ferlini
Alessandra
A
Cavo
Michele
M
Merlini
Giampaolo
G
Perlini
Stefano
S
Pasquali
Sonia
S
Branzi
Angelo
A
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
D012739:Gonadal Steroid Hormones