Free-wall right ventricular longitudinal strain discriminated AL amyloidosis from other etiologies (AUC 0.744) and independently predicted overall mortality (HR 1.113; 95% CI 1.029-1.204; p=0.007).
Observational (n=315)
Does free-wall right ventricular longitudinal strain (FWRVLS) help diagnose AL amyloidosis and predict overall mortality in patients with increased wall thickness?
Free-wall right ventricular longitudinal strain is a useful echocardiographic marker for both differentiating AL amyloidosis from other causes of increased wall thickness and predicting overall mortality.
Effect estimate: HR 1.113 (95% CI 1.029-1.204)
p-value: p=0.007
OBJECTIVES: Light-chain (AL) amyloidosis can lead to an infiltrative cardiomyopathy with increased wall thickness (IWT) of very poor prognosis. Our primary aim was to analyse the right ventricle (RV) in patients with IWT to discriminate AL amyloidosis from IWT due to hypertrophic cardiomyopathy (HCM) or to arterial hypertension (HTN). Our secondary aim was to assess if RV dysfunction predicts overall mortality in cardiac AL amyloidosis. METHODS: We retrospectively and consecutively compared clinical, biological and echocardiographic data of 315 patients with IWT: 105 biopsy-proven AL amyloidosis patients, 105 patients with HCM and 105 patients with HTN. The prognostic value of these parameters was analysed in the AL amyloidosis group. RESULTS: Free-wall right ventricular longitudinal strain (FWRVLS) worse than -21.2% discriminates AL amyloidosis area under the curve (AUC) = 0.744) from patients with IWT due to other aetiologies. In AL amyloidosis, FWRVLS is the strongest echocardiographic prognostic marker with AUC =0.722 and -16.5% as the optimal cut-off value, beyond which overall mortality increases significantly. It is also the only independent echocardiographic predictor of overall mortality (HR =1.113; 95%CI 1.029-1.204; p = .007), even when adjusted to the Mayo stage and global left ventricular longitudinal strain. CONCLUSIONS: FWRVLS should be considered in the diagnostic and prognostic workup in light-chain amyloidosis.
Uzan et al. (Wed,) conducted a observational in Light-chain (AL) amyloidosis with increased wall thickness (n=315). Free-wall right ventricular longitudinal strain (FWRVLS) vs. Hypertrophic cardiomyopathy or arterial hypertension was evaluated on Discrimination of AL amyloidosis from other etiologies and overall mortality (HR 1.113, 95% CI 1.029-1.204, p=0.007). Free-wall right ventricular longitudinal strain discriminated AL amyloidosis from other etiologies (AUC 0.744) and independently predicted overall mortality (HR 1.113; 95% CI 1.029-1.204; p=0.007).
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