PMC8035018_01
Electrocardiogram obtained on admission. Low voltage is noted in leads I, AVL; however, no left ventricular hypertrophy is noted. Q waves are seen in leads V1 and V2.
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fig1
A 12-lead electrocardiogram (ECG) obtained on presentation revealed normal sinus rhythm, low QRS voltage, poor R-wave progression, and Q waves in anteroseptal leads (Figure 1).
4-chamber (a) and a parasternal long axis echocardiogram view. Significant increased left ventricular wall thickness and biatrial enlargement can be seen.
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fig2
There was abnormal global longitudinal (GL) strain with apical longitudinal strain preservation, a pattern suggestive of cardiac amyloidosis (CA) (Figures 2 and 3).
Decreased global longitudinal strain pattern on echocardiogram. There is apical sparing of longitudinal strain, a finding typically seen in cardiac amyloidosis.
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fig3
There was abnormal global longitudinal (GL) strain with apical longitudinal strain preservation, a pattern suggestive of cardiac amyloidosis (CA) (Figures 2 and 3).
Left anterior oblique caudal images from left heart catheterization. The right coronary system (a) and left coronary system (b) appear angiographically normal.
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This showed angiographically normal coronaries (Figure 4) and an LVEDP of 12-14 mmHg.
Delayed gadolinium enhancement cardiac MRI images. The image on (a) shows a short axis view, (b) is a 4-chamber view. Extensive midmyocardial and atrial wall enhancement secondary to amyloid deposition is noted (green arrows) which is a signature of cardiac amyloidosis.
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fig5
This was also noted throughout the left atrium and along the septal subendocardial surface of the right ventricle (Figure 5).
Pyrophosphate scan (99mTc-PYP). Increased cardiac uptake can be seen in the image in (a) denoted by purple circle.
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TTR amyloidosis (Figure 6). The heart to contralateral (H/CL) ratio was 1.41.
Amyloidosis in abdominal fat. Abdominal fat pad biopsy revealed pretentious infiltrate (arrow) which shows apple-green birefringence on red stain, consistent with amyloidosis (H&E stain, 100x).
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Fat pad aspirate biopsy showed positive staining for Congo red (Figure 7).