PMC5337461_01
12-Lead electrocardiogram for 8 years. Incomplete left bundle branch block appeared in 2009 and progressed to complete left bundle branch block in 2011. The QRS width further increased in 2015. The PR interval also widened over 8 years.
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His blood pressure was 120/60 mmHg with a regular pulse rate of 72 beats/min. A neurological examination revealed impairment of pain and temperature sensation in the distal lower limbs. Chest radiography showed cardiomegaly (cardiothoracic ratio, 56.5%) without congestion or pleural effusion. He had undergone an annual routine ECGs for 8 years at the previous clinic, except in 2010 and 2014 (Fig. 1).
Transthoracic echocardiography on admission. Transthoracic echocardiography showed concentric biventricular hypertrophy. A small amount of pericardial effusion was observed anterior to the right ventricle.
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Right ventricular hypertrophy and a small amount of pericardial effusion anterior to the right ventricle were also noted (Fig. 2).
A: Late gadolinium-enhanced cardiac magnetic resonance imaging. Characteristic global subendocardial and transmural pattern of myocardial late gadolinium enhancement is shown in the thickened wall of both ventricles, since the contrast agent distributed to the extracellular space expanded by amyloid infiltration. Note that, in cardiac amyloidosis, the blood pool is abnormally dark (white star), possibly reflecting fast blood pool washout of the contrast media. B: The 99mTc-pyrophosphate scintigraphic findings. Diffuse cardiac accumulation of 99mTc-pyrophosphate was observed.
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Late gadolinium-enhanced cardiac magnetic resonance imaging (MRI) showed a characteristic global subendocardial and transmural pattern of myocardial late gadolinium enhancement in the thickened wall of both ventricles (Fig. 3A). 99mTc-pyrophosphate scintigraphy revealed diffuse accumulation in the entire myocardium (Fig. 3B).
The pathological findings of a colonal specimen. A colonal biopsy specimen by colon fiber shows amyloid deposits on the submucosal arterioles stained by direct fast scarlet (A), showing apple green birefringence (B). Immunohistochemical staining for amyloidogenic transthyretin was positive (C).
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Instead, colonal biopsy was performed, which showed amyloid deposits on the submucosal arterioles stained by direct fast scarlet (Fig. 4A), showing apple green birefringence (Fig. 4B). Immunohistochemical staining for ATTR was positive (Fig. 4C).