The First Korean Clinical Evidence With Tafamidis in ATTR-CM

1/3 https://e-kcj.orgTransthyretin amyloid cardiomyopathy (ATTR-CM) was once considered a rare and irreversible cause of heart failure, characterized by progressive myocardial infiltration of transthyretin amyloid fibrils leading to cardiac stiffness and dysfunction. 1)However, with the introduction of disease-modifying therapies, it has gained increasing recognition within the cardiology community. 2)The transthyretin stabilizer tafamidis demonstrated significant reductions in mortality and morbidity in the ATTR-ACT trial. 3)Nevertheless, although several studies from Japan have described the clinical experience and treatment outcomes with tafamidis, 4) evidence specific to Koreans remains limited.In this issue of the Korean Circulation Journal, Shin et al. 5) report the first Korean experience with tafamidis therapy in patients with ATTR-CM.The investigators categorized patients into three groups: those not receiving tafamidis, those treated with 20 mg, and those treated with 80 mg daily.They evaluated serial echocardiographic and laboratory parameters over a 12-24-month follow-up.They also assessed the composite endpoint of all-cause mortality and worsening heart failure requiring hospitalization or urgent visits.This study provides important real-world evidence showing that tafamidis-treated patients experienced lower rates of adverse clinical events compared with untreated patients, with consistent findings across both dose groups.These results align with observations from the ATTR-ACT trial 3) and provide valuable region-specific evidence from Korea.Beyond clinical outcomes, the authors contribute novel echocardiographic data.Comprehensive strain analysis demonstrated slower functional decline in the left ventricle (LV), left atrium (LA), and right ventricle (RV) among tafamidis-treated patients.Particularly, the LA stiffness index and RV-pulmonary circulation coupling index appeared more sensitive to disease progression than conventional LV ejection fraction or LV global longitudinal strain.These findings highlight the utility of multi-chamber strain parameters as more dynamic and sensitive complementary measures for monitoring therapeutic response of ATTR-CM.However, these parameters, while physiologically appealing, require prospective validation in larger cohorts before their prognostic and therapeutic implications can be applied in routine clinical practice.The inclusion of an untreated group strengthens the interpretation of the results by allowing direct comparison with the natural course of the disease.Event rates were highest in