- Poster presentation
- Open Access
Thickness of the midventricular left ventricular wall is predictive of survival in different forms of cardiac amyloidosis
© aus dem Siepen et al; licensee BioMed Central Ltd. 2013
- Published: 30 January 2013
- Cardiac Magnetic Resonance
- Cardiac Magnetic Resonance Imaging
- Cardiac Amyloidosis
- Systemic Amyloidosis
Systemic amyloidosis is a disorder characterized by extracellular deposition of different insoluble protein fibrils in various organs leading to organ dysfunction. Cardiac involvement is associated with limited survival. We aimed to use cardiac magnetic resonance imaging (CMR) to identify structural and functional alterations related to the different forms of amyloidosis, e. g. hereditary and wild-type transthyretin (TTR) amyloidosis as well as light-chain (AL) amyloidosis.
In total, 130 patients (82 male, 38 female; mean age 60.7±1.1 years) with different forms of amyloidosis (AL n= 72, TTR n=58) were evaluated by two blinded experienced observers employing a Vector-ECG gated 1.5T whole-body CMR (Achieva Intera® Philips Medical Systems, Best, The Netherlands). The study included SSFP and gadolinium contrast delayed enhancement (CE-CMR) 2-,3-,4-chamber and short-axis planes. EDV, ESV, EF and myocardial mass were analyzed on a standard workstation (Philips Viewform). Regional wall thickness was analyzed in modified 16 segment AHA-model of the left ventricle as well as the interatrial wall and compared between the different forms of amyloidosis. Univariate and multivariate analysis were performed to define predictors of survival.
According to this analysis of a large cohort of patients with different forms of cardiac amyloidosis maximal thickness of LV wall in the midventricular segments appears to be predictive of survival in patients with amyloidosis. Further studies are needed to confirm these results in a larger independent patient cohort especially of the TTR type.
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