Multi-modality imaging in transthyretin amyloid cardiomyopathy

Abstract

Transthyretin amyloid (TTR) cardiomyopathy is a disease of insidious onset, which is often accompanied by debilitating neurological and/or cardiac complications. The true prevalence is not fully known due to its elusive presentation, being often under-recognized and usually diagnosed only late in its natural history and in older patients. Because of this, effective treatment options are usually precluded by multiple comorbidities and frailty associated with such patients. Therefore, high clinical suspicion with earlier and better detection of this disease is needed. In this review, the novel applications of multimodality imaging in the diagnostic pathway of TTR cardiomyopathy are explored. These include the complimentary roles of transthoracic echocardiography, cardiac magnetic resonance, nuclear scintigraphy and positron emission tomography in quantifying cardiac dysfunction, diagnosis and risk stratification. Recent advances in novel therapeutic options for TTR have further enhanced the importance of a timely and accurate diagnosis of this disease.

Keywords: Multimodality imaging, Cardiac amyloidosis, Transthyretin, Echocardiography, Cardiac magnetic resonance, Nuclear imaging

Core tip: Non-invasive diagnosis of transthyretin amyloid (TTR) cardiomyopathy is improving with significant developments in multiple imaging modalities available to date. A greater appreciation of the various strengths and limitations of these imaging modalities is vital, as is high clinical suspicion and timely investigation for the disease, which remains insidious and elusive. This is of particular relevance in light of emerging novel effective therapeutic options. This focused review aims to highlight the role of multimodality imaging in the diagnosis and risk stratification of patients with TTR cardiomyopathy.