Temporal trends in the prevalence, incidence, and mortality of cardiac amyloidosis in South Korea over 12 years.

Objectives: This study investigated the prevalence, incidence, and prognosis of cardiac amyloidosis (CA) in South Korea.

Methods: This retrospective nationwide population-based study used the Health Insurance Review and Assessment Service databases between 2008 and 2020. All patients diagnosed with amyloidosis were included, and those with a diagnosis of heart failure or cardiomyopathy were classified as having CA. Both the special code for amyloidosis (V121), which enables coverage of medical expenses, and the corresponding International Classification of Diseases, 10th Revision codes for amyloidosis (E850-E854, E858, E859) were used to improve the reliability of amyloidosis diagnosis.

Results: Among 2,239 patients with amyloidosis, 758 met the criteria for CA (mean age, 64.4±11.9 years; 59.1% male). The mean age of patients with CA increased from 59.5±14.7 years in 2009 to 68.1±13.9 years in 2020. The incidence and prevalence increased from 0.09 (95% confidence interval [CI], 0.06-0.12) to 0.22 (95% CI, 0.18-0.27) per 100,000 person-years and 0.20 (95% CI, 0.16-0.25) to 1.30 (95% CI, 0.12-0.42) per 100,000 persons, respectively (all p<0.001). Patients with light-chain CA showed similar trends. In-hospital mortality decreased from 17.3% (95% CI, 9.23%-29.6%) to 6.1% (95% CI, 4.21%-8.48%) between 2009 and 2020. While age-specific in-hospital mortality was significantly higher in patients aged ≥70 years (p=0.004), no significant age-specific difference in in-hospital mortality was observed in patients with CA aged <70 years (p=0.981).

Conclusion: The prevalence and incidence of CA have increased in South Korea, predominantly affecting older individuals, particularly males. Notably, in-hospital mortality decreased significantly.