Gender-related risk of myocardial involvement in systemic amyloidosis

Claudio Rapezzi, Letizia Riva, Cristina C. Quarta, Enrica Perugini, Fabrizio Salvi, Simone Longhi, Paolo Ciliberti, Francesca Pastorelli, Elena Biagini, Ornella Leone, Robin M T Cooke, Letizia Bacchi-Reggiani, Alessandra Ferlini, Michele Cavo, Giampaolo Merlini, Stefano Perlini, Sonia Pasquali, Angelo Branzi

Research output: Contribution to journalArticlepeer-review


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 (∼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.

Original languageEnglish
Pages (from-to)40-48
Number of pages9
Issue number1
Publication statusPublished - 2008


  • Amyloidosis
  • Cardiomyopathies
  • Female
  • Male

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Medicine(all)


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