The Evolving Role of Cardiovascular Magnetic Resonance Imaging in the Evaluation of Systemic Amyloidosis

Abstract

Systemic amyloidosis is a serious multiorgan disease with reduced life expectancy, irrespective of type. The impact of magnetic resonance imaging (MRI) in managing this condition has been immense. The last decade in particular has seen a surge of interest in the assessment and evaluation of the heart in patients with systemic amyloidosis by cardiovascular magnetic resonance imaging (CMR), with approximately 85% of all publications on this subject arising in the last 10 years. This has been largely driven by the creation of new sequences and their subsequent modernisation and technical development, thereby rendering previously prohibitive methods clinically more relevant and applicable. In turn, this has led to an increased awareness and recognition of the disease. This review demonstrates how MRI has become a pivotal diagnostic tool in the assessment of cardiac amyloidosis over the last 2 decades, with the ability to track disease and predict mortality. Several different pathognomonic patterns of late gadolinium enhancement (LGE) are now recognised and are able to prognosticate. T1 mapping and extracellular volume (ECV) techniques have resulted in even earlier disease detection before LGE is even visible and along with T2 mapping, provide new insights into biology. As newer therapies also evolve and become available, the need for accurate tracking of cardiac disease response to treatment carries increasing importance. All these are examined in this review, mainly focussing on light-chain (AL) and transthyretin (ATTR) amyloidosis.

Regional Amyloid Burden Differences Evaluated Using Quantitative Cardiac MRI in Patients with Cardiac Amyloidosis

“…Additionally, we compared the mapping and ECV values between different types of amyloidosis. The TTR type has lower T1 and higher ECV with a more benign disease course than the AL type [ 8 18 34 ]. In our study, ECV was slightly higher in the TTR group than in the AL group; however, there was no statistical significance.…”

“…However, LGE patterns are not always visible and may be atypical or patchy [ 5 6 7 ]. Moreover, LGE imaging is sometimes challenging in patients with CA owing to diffuse amyloid deposition throughout the heart, causing incorrect nulling [ 8 ]. CA shows a regional apical sparing pattern on 2-dimensional (2D) speckle tracking echocardiographic strain imaging [ 4 9 ].…”

Native T1 mapping and extracellular volume fraction for differentiation of myocardial diseases from normal CMR controls in routine clinical practice

“…Native T1 mapping also have benefit in the detection of disease in case of impaired renal function that prohibit the use of CMR contrast agents and cannot acquire LGE images. Besides, T1 and ECV changes can provide earlier disease detection, even before LGE is detected [ 28 ].…”

Diagnostic imaging of cardiac amyloidosis

“…In the past decade, CMR has emerged as a robust imaging technique that not only provides structural and functional data, but most importantly conveys valuable information regarding tissue composition. An exponential increase has been observed in the use of CMR to assess the hearts of patients with systemic amyloidosis, with approximately 85% of all publications on this subject arising in the past 10 years alone 58 . This increase has been largely driven by the emergence of new CMR sequences and their subsequent modernisation and technical development, which has led to an increased awareness and recognition of the cardiac amyloidosis.…”