Author + information
- Arnt Kristen,
- Fabian aus dem Sieben,
- Katrin Scherer,
- Rebekka Kammerer,
- Sebastian Buss,
- Evangelos Giannitsis,
- Hugo A. Katus and
- Henning Steen
Cardiac involvement is associated with limited survival in systemic amyloidosis. We aimed to use cardiac magnetic resonance imaging (CMR) to identify structural and functional alterations related to 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) including 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 AHA-16 segments of the LV and interatrial. Univariate and multivariate analysis were performed to define predictors of survival.
Patients with TTR amyloidosis were significantly older as compared to patients with AL. They had higher left ventricular mass (171.9±8.1g vs. 146.1±6.9g, p<0.01) and thickness of interatrial septum (7.3±0.3mm vs. 5.7±0.2mm; p<0.001) with highest LV mass and thickness of interatrial septum in patients with wild-type amyloidosis (n=26). Regional wall thickness was more pronounced in ATTR in the inferolateral and septal segments. In AL patients univariate analysis revealed median of maximal wall thickness of the basal (14 mm), midventricular (10 mm) and apical (7 mm) segments as predictors of survival. In multivariate analysis midventricular maximal wall thickness was the only independent predictor of survival. In TTR patients univariate analysis revealed median of maximal wall thickness of the midventricular (13.5 mm) and apical (8 mm), but not basal (16.0 mm) segments as predictors of survival. There was no independent predictor of survival in multivariate analysis of these patients.
According to this analysis maximal thickness of LV wall in the midventricular segments appears to be predictive of survival in patients with amyloidosis.
Poster Sessions, Expo North
Saturday, March 09, 2013, 3:45 p.m.-4:30 p.m.
Session Title: What Is Unfolding in Cardiac Amyloidosis Research?
Abstract Category: 23. Pericardial/Myocardial Disease
Presentation Number: 1163-141
- 2013 American College of Cardiology Foundation