Key result
Cardiac magnetic resonance imaging detected cardiac involvement in 60% of patients with hereditary ATTR amyloidosis, and in 20% of subjects without left ventricular wall thickening.
Why the study?
Does cardiac magnetic resonance (CMR) imaging detect preclinical cardiac involvement in patients with hereditary ATTR amyloidosis and asymptomatic carriers?
Observational (n=67)
Does cardiac magnetic resonance (CMR) imaging detect preclinical cardiac involvement in patients with hereditary ATTR amyloidosis and asymptomatic carriers?
CMR imaging can detect preclinical cardiac involvement in patients with hereditary ATTR amyloidosis, even in those with isolated neurological symptoms or without left ventricular wall thickening.
May support CMR screening for preclinical cardiac involvement in hereditary ATTR; leaves open impact on outcomes and need for prospective validation.
BACKGROUND: Most of the studies that described cardiac amyloidosis using cardiac magnetic resonance (CMR) imaging refer to patients with primary light chain (AL) amyloidosis. The goal of this study was to evaluate cardiac involvement in patients with hereditary transthyretin associated (ATTR) amyloidosis and asymptomatic carriers and its relationships with clinical symptoms and genotype, using CMR imaging. METHODS AND RESULTS: Fifty-three patients with hereditary ATTR amyloidosis and 14 asymptomatic carriers were included in this study. Morphological, functional and late gadolinium enhancement (LGE) findings were noted on CMR images. A positive LGE suggesting cardiac amyloidosis was detected in 60% of patients. The pattern of LGE was diffuse, focal and circumferential in 32, 26 and 2% of patients, respectively. The inferior basal segment was the most frequently involved (93%) in case of focal involvement. Diffuse pattern was exclusively encountered in patients with cardiac symptoms. Nineteen percent of patients with isolated neurological symptoms and 20% of subjects without left ventricular wall thickening exhibited cardiac abnormalities on CMR. CONCLUSION: Cardiac involvement can be detected in patients with hereditary ATTR amyloidosis with isolated neurological symptoms and without left ventricular wall thickening, suggesting that CMR could be useful in detecting preclinical cardiac amyloidosis.
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Deux et al. (2014) conducted an observational in Hereditary transthyretin associated (ATTR) amyloidosis (n=67). Cardiac magnetic resonance (CMR) imaging was evaluated on Positive late gadolinium enhancement (LGE) suggesting cardiac amyloidosis. Cardiac magnetic resonance imaging detected cardiac involvement in 60% of patients with hereditary ATTR amyloidosis, and in 20% of subjects without left ventricular wall thickening.
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