Cardiac amyloidosis

Case contributed by Dr Tim Luijkx

Presentation

Over last two years underwent analysis for pain in upper abdomen, 31 kg weight loss, diarrhoea; progressive axonal sensomotoric polyneuropathy with autonomous symptoms. M protein negative, TTR mutation analysis ongoing, light chains elevated. High clinical suspicion of amyloidosis.

Patient Data

Age: 60
Gender: Male
Modality: MRI

Patient characteristics:
Length 184 cm, weight 77 kg, BSA 2,00 m²

Diameters:
- LV: 46 mm
- RV: 44 mm
- Septum: 18 mm, lateral wall 10 mm, anterior wall 14 mm, inferior wall 13 mm

Functional analyses:
Left ventricle:
- EDV: 187 mL (94 mL/m²)
- ESV: 75 mL (37 mL/m²)
- SV: 113 mL (56 mL/m²)
- EF: 60%
- CO: 6.6 L/min (3.3 L/min/m²)

Right ventricle:
- EDV: 170 mL (85 mL/m²)
- ESV: 69 mL (34 mL/m²)
- SV: 101 mL (51 mL/m²)
- EF: 60%
- CO:  5.9 L/min (3.0 L/min/m²)

Concentric hypertrophy of LV wall, most prominent septally. Enlarged atria. No signs of LV outflow tract obstruction or systolic anterior motion (SAM) of the mitral valve. Synchronous contraction of both ventricles. No focal wall motion abnormalities. No valvular insufficiencies. No signs of iron deposition (T2* value 23 ms). 

On early and late enhancement series diffuse enhancement of the myocardium with concentric subendocardial apical and mid-ventricular ring enhancement in the late phase. There is also enhancement of papillary muscles, RV wall and both atria. Low signal intensity of the blood pool compared to myocardium. Pericardial effusion. Bilateral pleural effusion. Haemangioma in vertebral body T8.

Conclusion:
Images in keeping with cardiac amyloidosis.

Case Discussion

Although there seems to be concentric left ventricular hypertrophy, this would be a misnomer as there is merely thickening of the LV wall due to amyloid deposition in the extracellular space. The myocardium itself is not hypertrophied. The amyloid results in a reduced capability of the heart to contract (restrictive pattern). 

Also note that there is reduced signal intensity of the blood pool due to pooling of contrast in the enlarged extracellular space of the ventricular walls.

Cardiac amyloidosis carries a very poor prognosis.  

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Case Information

rID: 39736
Case created: 17th Sep 2015
Last edited: 11th Nov 2015
System: Cardiac
Inclusion in quiz mode: Included

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