Korean Circ J.  2024 Apr;54(4):221-222. 10.4070/kcj.2024.0022.

Cardiomyopathy Without Amyloid Deposit in Systemic Light Chain Deposition Disease

• Park BE
• Jang SY
• Park YJ
• Bae MH
• Lee JH
• Yang DH
• Park HS
• Cho Y

• KMID: 2555535
• DOI: http://doi.org/10.4070/kcj.2024.0022

Figure

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  Figure 1 Images and histological analyses in a case with cardiac light chain deposition disease. (A) Transthoracic echocardiographic image showing left ventricular wall thickness with disproportionate interventricular septal enlargement, resulting in a ‘speckled’ appearance and small pericardial effusion (parasternal long-axis view). (B) Regional longitudinal strain, assessed by 2-dimensional speckle-tracking echocardiography. The reduction in global longitudinal strain is particularly observed in the basal and mid-ventricular segments, manifesting as an ‘apical sparing’ pattern. The mean global longitudinal strain was −8.5%. (C) Electrocardiogram; The electrocardiogram of the patient reveals characteristic findings, including low voltage in the limb leads (QRS amplitude <5 mm in leads II, III, aVR, aVF) and a pseudo-infarct pattern (pathologic Q waves in V1, V2, V3, accompanied by the loss of R wave progression). (D, G) Histological analyses of endomyocardial biopsy specimens. (D) Trichrome staining: The blue areas signify significant interstitial fibrosis, original magnification ×100. (E) Congo red staining; The Congo red stain did not show extracellular deposition of amyloid fibrils in the myocardial interstitium, original magnification ×100. (F) Immunofluorescence using anti-κ light chain antibody; the myocardial interstitium shows a negative reaction, original magnification ×100. (G) Immunofluorescence using anti-λ light chain antibody; The immunostaining with anti-λ light chain antibodies reveals intense positivity in the myocardial interstitium, original magnification ×100.aVR = augmented vector right; aVF = augmented vector foot.

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