PMC5829326_01
EKG demonstrating low-voltage criteria in most of the leads. Although the influence of the pericardial effusion is uncertain, this finding could be consistent with an infiltrative disease.
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Her EKG showed sinus rhythm, signs of left atrial enlargement and low voltage in the majority of leads (Figure 1).
Transthoracic echocardiogram showing moderate-size pericardial effusion in both parasternal short axis view ((a) systole; (b) diastole) and parasternal long axis view ((c) systole; (d) diastole). Echocardiogram also demonstrates granular sparkling (speckled) appearance of myocardium, suggestive of cardiac amyloid.
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A transthoracic echocardiogram demonstrated a large pericardial effusion, left ventricular ejection fraction of 73%, grade I diastolic dysfunction, left ventricular hypertrophy, abnormal longitudinal contraction, and a granular sparkling (speckled) appearance of the myocardium, findings suggestive of ACM (Figure 2).
Bone marrow biopsy. Congo red 100x. Evidence of amyloid deposits.
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Congo red positive amyloid deposits, and mass spectrometry consistent with AL (lambda)-type amyloidosis (Figure 3). Cardiac magnetic resonance (CMR) demonstrated suboptimal myocardial nulling with patchy focal late gadolinium enhancement affecting the pericardium and atrial walls, a large pericardial effusion, and a pleural effusion suggestive of amyloid infiltration (Figure 4).
Cardiac MRI. Evidence of patchy focal late gadolinium enhancement of the pericardium with suboptimal nulling of the myocardium.
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fig4
Congo red positive amyloid deposits, and mass spectrometry consistent with AL (lambda)-type amyloidosis (Figure 3). Cardiac magnetic resonance (CMR) demonstrated suboptimal myocardial nulling with patchy focal late gadolinium enhancement affecting the pericardium and atrial walls, a large pericardial effusion, and a pleural effusion suggestive of amyloid infiltration (Figure 4).
Pericardial biopsy. Congo red under polarized light 200x. Evidence of apple-green birefringence indicative of the presence of amyloid fibrils.
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Results of this biopsy were consistent with deposition of amyloid, confirming the diagnosis of ACM (Figure 5).