Diagnostic score for the detection of cardiac amyloidosis in patients with left ventricular hypertrophy and impact on prognosis

AbstractBackground: Among diagnosis associated with left ventricular hypertrophy (LVH), cardiac amyloidosis (CA) is a progressive disease with poor prognosis. Early noninvasive identification is of growing clinical importance. The objective of our study was to integrate clinical, biologic, electrocardiographic and echocardiographic parameters to build a diagnostic score in patients with LVH.Methods and results: One hundred and fourteen patients with LVH underwent a cardiac magnetic resonance (CMR) and a 99mTc-hydroxymethylene-diphosphonate scintigraphy (99mTc-HMDP) allowing to discriminate three groups of diagnoses: CA (n = 50 including 31, 18 and 1 ATTR, AL and AA amyloidosis), hypertrophic cardiomyopathy (n = 19) and unspecific cardiomyopathy (n = 45). Seven continuous variables associated with CA (systolic arterial pressure 200 ms; Sokolow index 13 mm; E/Ea ratio >10; global longitudinal strain > −12% and sum of basal longi...