Amyloidosis

Buzz

  • The hallmark of CA  is
  • LGE
    • Difficult to null
    • Subendocardial
    • Apical sparing
    • Atrial
  • Enlargement
    • Increased LV thickness but
      • can be LV and RV and
    • Biatrial dilatation
A INSIDE the HEART is for CONGO RED AMYLOIDOSIS
The hallmark of cardiac amyloidosis  is
LGE involving subendocardial regions with apical sparing
Ashley Davidoff MD
CARDIAC MUSCLE INFILTRATION OF CONGO RED AMYLOIDOSIS
In cardiac amyloidosis increased LV thickness is common , but may involve RV and atrial septum with bilateral atrial enlargement.
Ashley Davidoff MD

 

 

AL = hereditary =aggressive – heart involved in 50%
AA = acquired

Extracellular space often increases due to amyloid deposition.

LGE patterns

subendocardial

 patchy,

diffuse or

transmural (poorer prognosis)

Cardiac Amyloid                                                                                                                                                              MRI in Short Axis projection following Gd injection
82 year old female with history of diastolic heart failure
Top left – Atrial walls show  delayed enhancement
Top right – LGE in subendocardial regions of LV and RV
Bottom left – LGE in papillary muscles and subendocardial regions
Bottom right LGE in papillary muscles and subendocardial regions
Courtesy Ashley Davidoff MD
heart-00063
Cardiac Amyloid
82 year old female
with diastolic heart failure
Delayed enhancement is noted in the walls of both atria and a resulting zebra appearance (white black white) of the interatrial septum. LGE also noted in the
papillary muscle in the subendocardial regions of the LV and RV
Ashley Davidoff MD
heart-00064
Cardiac Amyloid
82 year old female
with diastolic heart failure
4 chamber view shows left ventricular hypertrophy and left effusion and mild enlargement of the left atrium
Ashley Davidoff MD
heart-00066
BIVENTRICULAR INVOLVEMENT
Late gadolinium enhancement in amyloidosis. Because certain artefacts regarding acquisition timing may occur when acquiring late gadolinium enhancement (LGE), the preferred technique to do LGE in amyloidosis is phase-sensitive inversion recovery (PSIR). PSIR allows to characterise better the segments that have gadolinium enhancement. The characteristic pattern is ‘global endocardial’, which also has been described as having transmural and ‘patchy’ characteristics. This case illustrated above looks mostly transmural and patchy. Note biventricular thickening
Agha, A et al Role of cardiovascular imaging for the diagnosis and prognosis of cardiac amyloidosis Open Heart BMJ
Cases on TCV

See Case 006

References and Links

Bejar, David et al. “Infiltrative Cardiomyopathies.” Clinical Medicine Insights. Cardiology vol. 9,Suppl 2 29-38. 8 Jul. 2015, doi:10.4137/CMC.S19706

Selvanayagam et al Evaluation and Management of the Cardiac Amyloidosis JACC Volume 50, Issue 22, November 2007