Key result
Diffuse septal subendocardial late gadolinium enhancement on cardiac magnetic resonance demonstrated 88% sensitivity and 100% specificity in diagnosing cardiac AL-amyloidosis.
Why the study?
Detection of cardiac AL amyloidosis is challenging and limited CMR data are available, despite its role in differentiating cardiomyopathies.
Does cardiac magnetic resonance (CMR) accurately differentiate cardiac light chain (AL) amyloidosis from hypertrophic cardiomyopathy and arterial hypertension?
Observational (n=435)
No
Does cardiac magnetic resonance (CMR) accurately differentiate cardiac light chain (AL) amyloidosis from hypertrophic cardiomyopathy and arterial hypertension?
Absolute Event Rate: 88% vs 100%
CMR provides high diagnostic accuracy for differentiating cardiac AL-amyloidosis from hypertrophic cardiomyopathy and hypertensive heart disease, primarily driven by the presence of diffuse subendocardial LGE and abnormal gadolinium kinetics.
May aid noninvasive diagnosis of cardiac AL-amyloidosis; leaves open need for prospective validation before clinical adoption.
BACKGROUND: While patients with cardiac transthyretin amyloidosis are easily diagnosed with bone scintigraphy, the detection of cardiac light chain (AL) amyloidosis is challenging. Cardiac magnetic resonance (CMR) analyses play an essential role in the differential diagnosis of cardiomyopathies; however, limited data are available from cardiac AL-Amyloidosis. Hence, the purpose of the present study was to analyze the potential role of CMR in the detection of cardiac AL-amyloidosis. METHODS: We included 35 patients with proved cardiac AL-amyloidosis and two control groups constituted by 330 patients with hypertrophic cardiomyopathy (HCM) and 70 patients with arterial hypertension (HT), who underwent CMR examination. The phenotype and degree of left ventricular (LV) hypertrophy and the amount and pattern of late gadolinium enhancement (LGE) were evaluated. In addition, global and regional LV strain parameters were also analyzed using feature-tracking techniques. Sensitivity and specificity of several CMR parameters were analyzed in diagnosing cardiac AL-amyloidosis. RESULTS: The sensitivity and specificity of diffuse septal subendocardial LGE in diagnosing cardiac AL-amyloidosis was 88% and 100%, respectively. Likewise, the sensitivity and specificity of septal myocardial nulling prior to blood pool was 71% and 100%, respectively. In addition, a LV end-diastolic septal wall thickness ≥ 15 mm had an optimal diagnostic performance to differentiate cardiac AL-amyloidosis from HT (sensitivity 91%, specificity 89%). On the other hand, a reduced global LV longitudinal strain (< 15%) plus apical sparing (apex-to-base longitudinal strain > 2) had a very low sensitivity (6%) in detecting AL-Amyloidosis, but with very high specificity (100%). CONCLUSIONS: The findings from this study suggest that CMR could have an optimal diagnostic performance in the diagnosis of cardiac AL-amyloidosis. Hence, further larger studies are warranted to validate the findings from this study.
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Dohy et al. (2022) conducted an observational in Cardiac light chain (AL) amyloidosis (n=435). Cardiac magnetic resonance (CMR) imaging vs. Hypertrophic cardiomyopathy and arterial hypertension was evaluated on Diagnostic sensitivity and specificity of diffuse septal subendocardial late gadolinium enhancement (LGE) for cardiac AL-amyloidosis. Diffuse septal subendocardial late gadolinium enhancement on cardiac magnetic resonance demonstrated 88% sensitivity and 100% specificity in diagnosing cardiac AL-amyloidosis.
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