Wild-Type Vs. Hereditary ATTR-CM Phenotypes1-3

Transthyretin Amyloid Cardiomyopathy   >   ATTR-CM   >   Pathogenesis, Subtypes and Diagnosis   >   ATTR-CM Subtypes

Wild-type and hereditary patients may have phenotypic differences,
but there can also be significant overlap.

WILD-TYPE
  • Idiopathic nonhereditary disease1,2
  • Thought to be the most common form of ATTR-CM3
  • Predominantly affects older men of white ethnicity1,3-6
  • Symptom onset at ~60 years of age7 and prevalence increases with age7,8
  • Patients may have cardiac arrhythmias, particularly atrial fibrillation1,3,6,9
  • History of carpal tunnel syndrome, lumbar spinal stenosis, neurological symptoms, and/or gastrointestinal symptoms1,6,10
 Median survival without treatment: ~3.5 years after diagnosis1,6,11
HEREDITARY‡
  • Due to TTR gene mutation, over 120 known mutations2,12
  • Autosomal dominant inheritance pattern3
  • Affects men and women3, and the geographic/ethnic distribution varies by mutation7,9
    • African American, African, or Afro-Caribbean descent (V122I)3,9
    • Irish descent (T60A)9
  • Symptom onset varies according to mutation, but may occur as early as 50 to 60 years of age3
  • History of neurological symptoms, gastrointestinal symptoms, and/or carpal tunnel syndrome3
 Median survival without treatment: ~2-3 years after diagnosis9