PMID- 31434577 OWN - NLM STAT- MEDLINE DCOM- 20200504 LR - 20240117 IS - 1532-429X (Electronic) IS - 1097-6647 (Print) IS - 1097-6647 (Linking) VI - 21 IP - 1 DP - 2019 Aug 22 TI - Prognostic value of novel imaging parameters derived from standard cardiovascular magnetic resonance in high risk patients with systemic light chain amyloidosis. PG - 53 LID - 10.1186/s12968-019-0564-1 [doi] LID - 53 AB - BACKGROUND: The differentiated assessment of functional parameters besides morphological changes is essential for the evaluation of prognosis in systemic immunoglobulin light chain (AL) amyloidosis. METHODS: Seventy-four subjects with AL amyloidosis and presence of late gadolinium enhancement (LGE) pattern typical for cardiac amyloidosis were analyzed. Long axis strain (LAS) and myocardial contraction fraction (MCF), as well as morphological and functional markers, were measured. The primary endpoint was death, while death and heart transplantation served as a composite secondary endpoint. RESULTS: After a median follow-up of 41 months, 29 out of 74 patients died and 10 received a heart transplant. Left ventricular (LV) functional parameters were reduced in patients, who met the composite endpoint (LV ejection fraction 51% vs. 61%, LAS - 6.9% vs - 10%, GLS - 12% vs - 15% and MCF 42% vs. 69%; p < 0.001 for all). In unadjusted univariate analysis, LAS (HR = 1.05, p < 0.001) and MCF (HR = 0.96, p < 0.001) were associated with reduced transplant-free survival. Kaplan-Meier analyses showed a significantly lower event-free survival in patients with reduced MCF. MCF and LAS performed best to identify high risk patients for secondary endpoint (Log-rank test p < 0.001) in a combined model. Using sequential Cox regression analysis, the addition of LAS and MCF to LV ejection fraction led to a significant increase in the predictive power of the model (chi(2) (df = 1) = 28.2, p < 0.001). CONCLUSIONS: LAS and MCF as routinely available and robust CMR-derived parameters predict outcome in LGE positive AL amyloidosis. Patients with impaired LV function in combination with reduced LAS and MCF are at the highest risk for death and heart transplantation. FAU - Arenja, Nisha AU - Arenja N AD - Department of Cardiology, Angiology and Pneumology, University of Heidelberg, Im Neuenheimer Feld 410, 69120, Heidelberg, Germany. nisha_arenja@yahoo.de. AD - Department of Cardiology, Kantonsspital Olten, Solothurner Spitaler AG, Baslerstrasse 150, 4600, Olten, Switzerland. nisha_arenja@yahoo.de. FAU - Andre, Florian AU - Andre F AD - Department of Cardiology, Angiology and Pneumology, University of Heidelberg, Im Neuenheimer Feld 410, 69120, Heidelberg, Germany. FAU - Riffel, Johannes H AU - Riffel JH AD - Department of Cardiology, Angiology and Pneumology, University of Heidelberg, Im Neuenheimer Feld 410, 69120, Heidelberg, Germany. FAU - Siepen, Fabian Aus dem AU - Siepen FAD AD - Department of Cardiology, Angiology and Pneumology, University of Heidelberg, Im Neuenheimer Feld 410, 69120, Heidelberg, Germany. FAU - Hegenbart, Ute AU - Hegenbart U AD - Department of Hematology, Oncology and Rheumatology, University of Heidelberg, Im Neuenheimer Feld 410, 69120, Heidelberg, Germany. FAU - Schonland, Stefan AU - Schonland S AD - Department of Hematology, Oncology and Rheumatology, University of Heidelberg, Im Neuenheimer Feld 410, 69120, Heidelberg, Germany. FAU - Kristen, Arnt V AU - Kristen AV AD - Department of Cardiology, Angiology and Pneumology, University of Heidelberg, Im Neuenheimer Feld 410, 69120, Heidelberg, Germany. FAU - Katus, Hugo A AU - Katus HA AD - Department of Cardiology, Angiology and Pneumology, University of Heidelberg, Im Neuenheimer Feld 410, 69120, Heidelberg, Germany. AD - DZHK (German Centre for Cardiovascular Research), Partner Site Heidelberg/Mannheim, 69120, Heidelberg, Germany. FAU - Buss, Sebastian J AU - Buss SJ AD - Department of Cardiology, Angiology and Pneumology, University of Heidelberg, Im Neuenheimer Feld 410, 69120, Heidelberg, Germany. AD - Das Radiologische Zentrum, Radiology Center Sinsheim-Eberbach-Erbach-Walldorf-Heidelberg, Alte Waibstadter Str. 2a, 74889, Sinsheim, Germany. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20190822 PL - England TA - J Cardiovasc Magn Reson JT - Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance JID - 9815616 RN - 0 (Contrast Media) RN - K2I13DR72L (Gadolinium DTPA) SB - IM MH - Aged MH - Cardiomyopathies/*diagnostic imaging/mortality/physiopathology/surgery MH - Contrast Media/administration & dosage MH - Disease Progression MH - Female MH - Gadolinium DTPA/administration & dosage MH - Heart Transplantation MH - Humans MH - Immunoglobulin Light-chain Amyloidosis/*diagnostic imaging/mortality/physiopathology/surgery MH - *Magnetic Resonance Imaging MH - Male MH - Middle Aged MH - Myocardial Contraction MH - Predictive Value of Tests MH - Prognosis MH - Retrospective Studies MH - Risk Assessment MH - Risk Factors MH - Stroke Volume MH - Time Factors MH - Ventricular Function, Left PMC - PMC6704553 OTO - NOTNLM OT - Cardiovascular magnetic resonance OT - Immunoglobulin light chain amyloidosis OT - Long axis strain OT - Longitudinal function OT - Myocardial contraction fraction OT - Prognosis COIS- The authors declare that they have no competing interests. EDAT- 2019/08/23 06:00 MHDA- 2020/05/06 06:00 PMCR- 2019/08/22 CRDT- 2019/08/23 06:00 PHST- 2019/01/02 00:00 [received] PHST- 2019/07/23 00:00 [accepted] PHST- 2019/08/23 06:00 [entrez] PHST- 2019/08/23 06:00 [pubmed] PHST- 2020/05/06 06:00 [medline] PHST- 2019/08/22 00:00 [pmc-release] AID - S1097-6647(23)00226-0 [pii] AID - 564 [pii] AID - 10.1186/s12968-019-0564-1 [doi] PST - epublish SO - J Cardiovasc Magn Reson. 2019 Aug 22;21(1):53. doi: 10.1186/s12968-019-0564-1.