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Comparison of right ventricular involvement in AL and transthyretin-type cardiac amyloidosis by cardiovascular magnetic resonance
Journal of Cardiovascular Magnetic Resonance volume 11, Article number: P54 (2009)
Background
Increased right ventricular (RV) wall thickness and RV dysfunction are adverse prognostic signs in primary (AL) cardiac amyloidosis and suggest more extensive disease. RV involvement in other forms of cardiac amyloidosis remains poorly understood. We sought to determine differences in RV involvement for those with transthyretin (TTR) type and light-chain (AL) type cardiac amyloidosis using cardiovascular magnetic resonance (CMR).
Methods
We assessed a multi-center cohort of subjects with proven cardiac amyloidosis due to AL (n = 21, 14 M, age 61 +/- 11.5 yr) and TTR (n = 14 12 M, age 73 +/- 4.8 yr) using conventional cine SSFP CMR. RV thickening was defined as a wall thickness = 6 mm. Volumetric assessments of RV free wall mass, volumes and ejection fraction (EF) were measured by an independent core lab blinded to patient data.
Results
The prevalence of RVH was greater in the TTR group (71% vs. 48% AL group, p = 0.01). Similarly, RV free wall mass was increased in the TTR subjects (55.4 ± 20.6 g vs 37.3 ± 15.4 g; p < 0.01). In addition, RV end-diastolic volume was greater in TTR amyloidosis (190 ± 39 g vs 138 ± 48 g; p < 0.01) while RVEF was lower in the TTR subjects (37 ± 11% vs 53 ± 13%; p < 0.001). There were no differences in the degree of heart failure symptoms in the TTR and AL groups: NHYA Class I: 4 vs 5; Class II/III: 10 vs 15; Class IV: 0 vs 1, respectively (p = 0.69).
Conclusion
These data suggest that, while similar in clinical heart failure symptoms, TTR amyloidosis appears to have more prevalent and extensive RV involvement than AL. The clinical impact of RV involvement in TTR amyloidosis remains to be determined.
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Open Access This article is published under license to BioMed Central Ltd. This is an Open Access article is distributed under the terms of the Creative Commons Attribution 2.0 International License (https://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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Appelbaum, E., Harrigan, C., Biller, L. et al. Comparison of right ventricular involvement in AL and transthyretin-type cardiac amyloidosis by cardiovascular magnetic resonance. J Cardiovasc Magn Reson 11 (Suppl 1), P54 (2009). https://doi.org/10.1186/1532-429X-11-S1-P54
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DOI: https://doi.org/10.1186/1532-429X-11-S1-P54
Keywords
- Right Ventricular
- Cardiovascular Magnetic Resonance
- Amyloidosis
- Heart Failure Symptom
- Cardiac Amyloidosis