The diagnostic value of multiparameter cardiovascular magnetic resonance for early detection of light-chain amyloidosis from hypertrophic cardiomyopathy patients

Figures

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  FIGURE 1. Representative images of different stages of light chain amyloidosis patients. (A) Congo red stain fluorescence staining of deltoid muscle of a Mayo I stage patient showing red fluorescent bright of amyloid deposit (×400). (B) Congo red stain birefringence of (A) under polarized light showing amyloid (×400). (C) Immunohistochemistry stains for lambda (+) and kappa (–) light chains in the deltoid muscle of a Mayo I stage patient show amyloid deposits (×400). (D) Congo red stain fluorescence staining of the liver of a Mayo III stage patient showing red fluorescent bright of amyloid deposit (×400). (E) Congo red stain fluorescence staining of bone marrow stroma of a Mayo III stage patient showing red fluorescent bright of amyloid deposit (×400). (F) Congo red stain fluorescence staining of the vascular wall of a Mayo III stage patient showing red fluorescent bright of amyloid deposit (×400).

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  FIGURE 2. The flowchart of light chain amyloidosis patient selection.

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  FIGURE 3. A healthy control subject, an HCM patient and three different stages of AL amyloid patients were shown from left to right and index top to bottom (4CH, SA, GRS, GCS, GLS, native T1, ECV, and ECV bullseye, respectively. (Column 1) A healthy control subject without negative LGE and GRS (47.55%), GCS (–21.47%), GLS (–17.95%), normal T1 (1,251 ± 5 ms) and ECV (30.1 ± 2.1%) for the left ventricular. (Column 2) An HCM patient showed patch LGE and GRS (59.03%), GCS (–15.31%), GLS (–5.61), increased native T1 (1331 ± 8.1 ms) and ECV (31.1 ± 3.2%) for the left ventricular. (Column 3) An AL patient of Mayo I showed patchy LGE and GRS (31.82%), GCS (–18.31%), GLS (–10.67), increased native T1 (1,324 ± 21 ms) and ECV (36.4 ± 3.1%) for the left ventricular. (Column 4) An AL patient of Mayo II showed slightly diffuse LGE at the myocardium and GRS (25.79%), GCS (–15.84%), GLS (–7.13%), increased native T1 (1,422 ± 3 ms) and ECV (38.9 ± 3.5%) for the left ventricular. (Column 5) An AL patient of Mayo III showed global extensive LGE at the myocardium and GRS (10.13%), GCS (–8,88%), GLS (–7.09%), increased native T1 (1,421 ± 57 ms) and ECV (57.0 ± 4.6%) for the left ventricular. 4CH, four-chamber;…

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  FIGURE 4. Multiparameter CMR (GRS, GCS, GLS of left ventricular, LVMT, native Tl, ECV values) in HA, HCM, AL amyloidosis patients with different Mayo stages. (A) AL CA patients showed a decrease in LV GRS strain (H: 45.2 ± 9.4%, HCM: 38.3 ± 12.0%, M I/II: 36.5 ± 10.9%, M III: 26.4 ± 10.7%; H vs. Mayo III, P < 0.001) as compared to healthy controls. (B) AL CA patients showed an increase in LV GCS strain (H: –21.2 ± 1.8%, HCM: –15.0 ± 3.2%, M I/II: –19.0 ± 3.6%, M III: –16.2 ± 1.7, HCM vs. M I/II, M I/II vs. M III, P = 0.02), as compared between them. (C) Mayo stage I/II AL patients showed an increase in the absolute value of LV GLS strain (H: –16.8 ± 1.2%, HCM: –9.5 ± 1.8%, M I/II: –11.9 ± 3.0%, M III: –9.9 ± 3.5, HCM vs. M I/II, P = 0.02), as compared to HCM. (D) HCM patients showed an increase in LVMWT (H: 6.1 ± 0.7 mm, HCM: 18.8 ± 3.3 mm, M I/II: 14.3 ± 2.9 mm, M III: 15.9 ± 2.3 mm, HCM vs. M I/II, P < 0.001), as compared to Mayo I/II AL patients. (E) AL patients showed an increase in native Tl mapping (H: 1,243.5 ± 29.85 ms, HCM: 1,318.2 ± 32.4 ms, M I/II: 1,334.9 ± 49.9 ms, M III: 1,442.7 ± 74.9 ms, H vs. M I/II, HCM vs. M III, Mayo I/II vs. Mayo III, P < 0.001, respectively), as…

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  FIGURE 5. Correlation between the Mayo stage, biochemical markers (cTnI and NT-proBNP), and all CMR-related parameters, including EF, EDVI, ESVI, EDMMI, LVMWT, Native Tl mapping, ECV mapping, GLS, GRS, and GCS). Both native Tl mapping, ECV mapping, GLS, GRS, and GCS were strongly correlated to the Mayo stages (r = 0.93, 0.93, 0.70, 0.76, 0.64, respectively). However, EF, EDVI, and ESVI showed no significant correlation. Correlation between continuous variables was assessed using Pearson’s r correlation, such as native Tl, ECV mapping and strain values, etc., and with categorical variables using Spearman P correlation, such as Mayo stage. cTnI, Cardiac Troponin I; NT-proBNP, N-terminal pro-B-type natriuretic peptide; EF, left ventricle ejection fraction; EDVI, left ventricle end-diastolic volume index; ESVI, left ventricle end-systolic volume index; EDMMI, left ventricle end-diastolic maximum mass index; LVMWT, left ventricle maximum wall thickness; ECV, extracellular volume; GCS, global circumferential strain; GLS, global longitudinal strain; GRS, global radial strain. Symbols: P, Spearman correlation coefficient; r, Pearson correlation coefficient; blue, positive correlation; red,…

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  FIGURE 6. Receiver operating characteristics curve for GLS, GCS, and ECV mapping and pairwise parameter for differentiating between Mayo I/II AL amyloidosis and HCM patients. As shown, a GCS < –16.4%, GLS < –11.6%, or ECV > 33.2% could predict Mayo I/II amyloidosis from HCM patients. Among them, the combined diagnostic performance of GLS, GCS, and ECV mapping can well distinguish Mayo I/II AL amyloidosis patients from hypertrophic cardiomyopathy (AUC = 0.969, Youden index = 0.813). HCM, hypertrophic cardiomyopathy patients; ECV, extracellular volume; GLS, global longitudinal strain; GRS, global radial strain; AUC, area under the curve.