A Delphi-based consensus study on the diagnosis and management of transthyretin amyloid cardiomyopathy in Asia Pacific
W Lin, Y H Lin, A Okada, C Y Khoo, E Paratz, H B Liew, J Hyun, J C Mohan, N Better, N Tahara, R Krittayaphong, T Yingchoncharoen, Y Y OonAbstract
Background
Transthyretin amyloid cardiomyopathy (ATTR-CM) is increasingly recognised in Asia Pacific (APAC); however, variations in healthcare infrastructure and resource availability across the region complicate the direct adoption of major international disease management guidelines.
Purpose
This study aimed to generate APAC-focused expert recommendations for the diagnosis, treatment and monitoring of ATTR-CM that address regional challenges, priorities and resource variations to improve early diagnosis, standardise care and optimise patient outcomes.
Methods
A modified Delphi approach was used to develop a set of consensus statements. Thirteen APAC-based cardiologists addressed 89 clinical questions across six domains: disease epidemiology and awareness; diagnosis, screening, staging, and prognosis; treatment strategies; monitoring disease progression and treatment response; long-term management; and future research priorities. The panellists participated in two Delphi rounds – Round 1 involved anonymous electronic surveys, while Round 2 comprised an in-person meeting for discussion and re-voting. Consensus was defined as ≥70% panel agreement.
Results
The expert panel achieved consensus on 60 statements. Key recommendations include screening patients aged ≥60 years with unexplained left ventricular wall thickness ≥12 mm and red flags; using bone scintigraphy with exclusion of amyloid light-chain amyloidosis as diagnostic standard; and performing single-photon emission computed tomography over planar imaging when available. For indicated disease-modifying therapy (DMT), lifelong treatment is recommended once initiated, with early initiation having strong prognostic impact. In addition to DMT, sodium-glucose cotransporter 2 inhibitors and mineralocorticoid antagonists should also be considered for heart failure management, while anticoagulation is recommended for atrial fibrillation regardless of cardiovascular risk scores. Multidomain assessment incorporating functional status, biomarkers and echocardiography should be considered every 6 months for monitoring purposes. The panel also advocates for better integration of updated screening recommendations into existing local clinical guidelines and improved access to specialised amyloidosis centres.
Conclusion
This Delphi consensus provides pragmatic recommendations for ATTR-CM management tailored to APAC contexts. Implementation of these recommendations may help standardise care delivery and improve outcomes across diverse healthcare settings in the region.