The Role of Multi-modality Imaging in the Diagnosis of Cardiac Amyloidosis: A Focused Update

Abstract

Cardiac amyloidosis (CA) is a unique disease entity involving an infiltrative process, typically resulting in a restrictive cardiomyopathy with diastolic heart failure that ultimately progresses to systolic heart failure. The two most common subtypes are light-chain and transthyretin amyloidosis. Early diagnosis of this disease entity, especially light-chain CA subtype, is crucial, as it portends a poorer prognosis. This review focuses on the clinical utility of the various imaging modalities in the diagnosis and differentiation of CA subtypes. This review also aims to highlight the key advances in each of the imaging modalities in the diagnosis and prognostication of CA.

Multimodality Cardiac Imaging in Cardiomyopathies: From Diagnosis to Prognosis

“…CMR provides high-resolution structural and functional information, allows tissue characterization [60], and permits diagnosis at early stages compared to TTE [63]. The main findings, in addition to those described by TTE, are the difficulty to null the myocardial signal in LGE sequences, a global or diffuse LGE, and a marked increase in native T1 and ECV values (>40%) (Figure 15) [60,63,64]. As the disease progresses, there is an increase in LGE uptake, which is initially subendocardial and progressively becomes transmural (which is associated with an increase in overall mortality regardless of the type of amyloidosis) [65].…”

“…Additionally, a recent meta-analysis showed that ECV is the strongest diagnostic and prognostic imaging biomarker in CA [66], and an ECV >58% is associated with increased mortality in ATTR [67]. CMR cannot distinguish ATTR and AL forms, but there are characteristically-associated signs (Table 4) [64,65,67,68]. The presence of a reduced GLS presents prognostic implications; a GLS >−14.8% has been associated with an increase in global mortality [61].…”

“…CMR provides high-resolution structural and functional information, allows tissue characterization [60], and permits diagnosis at early stages compared to TTE [63]. The main findings, in addition to those described by TTE, are the difficulty to null the myocardial signal in LGE sequences, a global or diffuse LGE, and a marked increase in native T1 and ECV values (>40%) (Figure 15) [60,63,64].…”

“…Additionally, a recent meta-analysis showed that ECV is the strongest diagnostic and prognostic imaging biomarker in CA [66], and an ECV >58% is associated with increased mortality in ATTR [67]. CMR cannot distinguish ATTR and AL forms, but there are characteristically-associated signs (Table 4) [64,65,67,68]. As the disease progresses, there is an increase in LGE uptake, which is initially subendocardial and progressively becomes transmural (which is associated with an increase in overall mortality regardless of the type of amyloidosis) [65].…”

Avaliação da disfunção miocárdica em modelo de choque hemorrágico controlado por meio de ecocardiografia 2D speckle tracking

“…[39][40][41] A análise de strain do VE também tem sido amplamente utilizada como ferramenta essencial para o diagnóstico diferencial de hipertrofia do VE associada à hipertensão arterial sistêmica, cardiomiopatia hipertrófica, estenose valvar aórtica, amiloidose cardíaca e doença de Fabry, por meio da análise do valor absoluto do SLG e da distribuição do padrão de comprometimento nos 17 segmentos miocárdicos. [42][43][44] Além disso, pesquisadores mostraram a importância da análise da deformidade miocárdica do átrio esquerdo por EET na avaliação CV de pacientes clinicamente saudáveis, com diabetes mellitus, hipertensão, insuficiência cardíaca e acidente vascular cerebral. [45][46][47] Neste estudo, o SLG do VE foi o único parâmetro de deformação miocárdica capaz de detectar a disfunção cardíaca subclínica associada ao ICIT, o que está de acordo com os achados de vários pesquisadores que demonstraram o valor prognóstico do strain longitudinal em pacientes com choque séptico [48][49][50] e com estudos populacionais que demostraram o strain longitudinal como preditor de incidência de insuficiência cardíaca, infarto agudo do miocárdio e doença cardiovascular.…”