Case Report: Contrast adenosine stress echocardiography revealing microvascular dysfunction in cardiac AL amyloidosis

Figures

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  Figure 1. Transthoracic echocardiogram showing typical echocardiographic features of cardiac amyloidosis: (A) parasternal long-axis view showing severe concentric left ventricular hypertrophy (white arrow), sparkling granular appearance of the myocardium, minimal pericardial effusion (yellow arrow), and left atrial(LA) enlargement. (B) Transmitral valve inflow diastolic peak early (E 0.82 m/s)/later (A 0.18 m/s) velocity obviously increased. (C) Tissue Doppler imaging showing markedly reduced septal early diastolic velocity of the mitral valve annular (e′ 3 cm/s). (D) Elevated tricuspid regurgitation velocity (TR 3.53 m/s) consistent with pulmonary hypertension.

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  Figure 2. Myocardial contrast echocardiography and coronary blood flow velocity assessment before and after adenosine infusion, consistent with impaired microvascular function and decreased blood flow reserve. (A–C) End-systolic perfusion by myocardial contrast echocardiography in apical four-chamber view. (A) Mild perfusion defect of LV part segments (arrows) before high-MI flash impulse. (B) Significant reduction in perfusion in the sixth sec post flash (arrows) before adenosine infusion. (C) Three minutes after adenosine infusion showing significant reduction in perfusion in the third sec post flash (arrows). (D,E) Coronary blood flow velocity spectrum of the mid left anterior descending artery (LAD). (C) Before adenosine infusion. (D) At the third minute after adenosine infusion. Reduced blood flow is seen with minimal systolic blood flow (white arrow), and increased diastolic blood flow resistance (yellow arrow).

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  Figure 3. Speckle-tracking strain imaging of left ventricle revealing reduced global longitudinal strain, with relative apical sparing, marked impairment at the base-midventricle (“cherry on the top” pattern), a characteristic feature of cardiac amyloidosis.

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  Figure 4. Renal biopsy (PAS stain, ×400) showing amorphous PAS-positive material within glomerular mesangium and small vessel walls, consistent with AL-type amyloid deposition.