Hereditary transthyretin amyloidosis (ATTRv) with polyneuropathy (ATTR-PN) is a rare genetic disorder.

Survival in patients with ATTR-PN is largely characterized by and dependent on associated cardiac damage, but the impact of non-cardiac characteristics on survival are less defined.

This study sought to assess the impact of non-cardiac baseline characteristics on survival in ATTR-PN.

Survival in patients with ATTR-PN was highly variable and affected by non-Val30Met mutation and non-cardiac baseline characteristics, such as autonomic dysfunction, large fiber involvement, and late-onset disease.