Patient Journey to Transthyretin Cardiac Amyloidosis Diagnosis - A Japanese Claims Database Study

Circ J. 2025 Mar 25;89(4):432-441. doi: 10.1253/circj.CJ-24-0666. Epub 2025 Jan 29.

Abstract

Background: Transthyretin amyloid cardiomyopathy (ATTR-CM) is an under-recognized cause of heart failure (HF) in older adults. Delayed ATTR-CM diagnosis may result in more advanced symptoms. This study describes the journey of Japanese patients with ATTR-CM.

Methods and results: This retrospective non-interventional study used the DeSC Healthcare database. Patients aged ≥18 years at the index date (date when ATTR-CM was first diagnosed or date of first tafamidis 80 mg prescription, whichever was earlier) and who had received ≥1 tafamidis 80 mg prescription or ≥1 specific ATTR-CM diagnosis, excepting "suspected diagnosis", at any time between April 1, 2014 and August 31, 2021 were included. The median age of patients was 79.0 years, and 79.9% (n=239) were male. The most frequently observed comorbidities defined as indicating the onset of ATTR-CM were HF (87.9%), atrial fibrillation/atrial flutter (50.2%), and conduction disorders (17.2%), with a median time from onset to index date of 15.5, 14.0, and 9.0 months for each comorbidity, respectively. Lumbar spinal stenosis (23.9%), neuropathy (13.0%), and carpal tunnel syndrome (7.5%) were common extracardiac symptoms, with a median time from the appearance of these symptoms to index date of 19.0, 5.0, and 18.0 months, respectively.

Conclusions: There was a delay between the appearance of cardiac and extracardiac comorbidities of ATTR-CM and its diagnosis in real-world Japanese clinical settings, emphasizing the need for early diagnosis of ATTR-CM.

Keywords: Japan; Patient journey; Tafamidis; Transthyretin amyloid cardiomyopathy.

MeSH terms

  • Aged
  • Aged, 80 and over
  • Amyloid Neuropathies, Familial* / complications
  • Amyloid Neuropathies, Familial* / diagnosis
  • Amyloid Neuropathies, Familial* / drug therapy
  • Amyloid Neuropathies, Familial* / epidemiology
  • Benzoxazoles / therapeutic use
  • Cardiomyopathies* / diagnosis
  • Cardiomyopathies* / drug therapy
  • Cardiomyopathies* / epidemiology
  • Comorbidity
  • Databases, Factual
  • East Asian People
  • Female
  • Heart Failure / diagnosis
  • Heart Failure / epidemiology
  • Heart Failure / etiology
  • Humans
  • Japan / epidemiology
  • Male
  • Middle Aged
  • Retrospective Studies

Substances

  • tafamidis
  • Benzoxazoles

Supplementary concepts

  • Amyloidosis, Hereditary, Transthyretin-Related
  • Japanese people