Cardiac amyloidosis: Upcoming challenges for appropriate diagnosis and treatment

Perceptions of transthyretin amyloid cardiomyopathy (ATTR-CM) have changed dramatically in recent years following the advent of contemporary cardiac imaging techniques and effective therapeutic options.

Recent studies are in agreement that early diagnosis and appropriate treatment are possible, with a positive impact on prognosis. Early recognition, followed by an appropriate multidisciplinary clinical approach, are fundamental steps in managing ATTR-CM.

The Working Group on Myocardial and Pericardial Diseases of the Portuguese Society of Cardiology have brought together a Task Force of experts on ATTR-CM and produced the first Portuguese recommendations on the screening, diagnosis and treatment of the disease, published in this supplement of the Portuguese Journal of Cardiology.1–3

With these important documents, we aim to provide clear information regarding current evidence-based guidelines for the clinical management of ATTR-CM, with emphasis on the importance of avoiding delay in diagnosis, initiation of TTR stabilizers to prevent amyloid aggregation (which are known to decrease death and cardiovascular-related hospitalizations in ATTR-CM), and treatment of clinical manifestations such as heart failure, thromboembolism, conduction disease and dysrhythmias.

Early referral to centers with the ability to provide a multidisciplinary approach, in which an interprofessional team collaborates in assessing and treating patients with ATTR-CM, is a pathway to defining the appropriate management of these patients and improving their outcomes. Increasing primary care physicians’ and cardiologists’ awareness to enable early identification of ATTR-CM is therefore paramount to ensure improvements in morbidity and mortality, and represents one of the clinical challenges in the field of ATTR-CM management.