Biventricular Thickening- Infiltration

  • What are the infiltrative cardiomyopathies that can cause biventricular infiltration
    • amyloidosis
    • sarcoidosis
    • Less commonly
      • Hemochromatosis
      • Fabry disease,
      • Dannon disease, and
      • Friedreich’s ataxia.
  • How do they affect function
    • primarily affect diastolic function and
    • less commonly systolic function
CARDIAC AMYLOIDOSIS with LV THICKENING – INFILTRATION VS LVH
66-year-old male presents with dyspnea
Non gated axial CT through the opening of the mitral valve suggests early diastole confirms concentric hypertrophy. The septum measures 24.1mms while the free wall measures 19.7mms. Upper limits normal is 14mms.
Ashley Davidoff MD

CARDIAC AMYLOIDOSIS
66-year-old male presents with dyspnea
Non gated sagittal CT through the RVOT shows RVH (right ventricular thickening) wall measuring between 6-7mm  involving both the RV inflow as well as the outflow See Case 006

CARDIAC AMYLOIDOSIS LGE SEQUENCE
Gated short axis delayed gadolinium sequence through the base LV during diastole and shows subendocardial LGE (red arrowheads in a,b,c, and d, diffuse mid myocardial LGE (white arrowheads) (a,b,c,d) and subepicardial LGE in the RV (yellow arrowheads (b,c)
Ashley Davidoff MD See Case 006
  • Key Issues for Cardiac Amyloid
    • The hallmark of CA
      • increased left ventricular thickness
      • but can be LV and RV and
      • BIatrial dilatation
      • Subendocardial LGE
      • Apical sparing
  • Difficult to null

 

Sarcoid Cardiomyopathy with Thickening of Both ventricles Due to Biventricular Infiltration

BI-VENTICULAR INFILTRATION WITH THICKENING
69 year old male presented with history of cardiomyopathy and atrial fibrillation
The LGE on the short axis shows bi-ventricular thickening of the RV and LV
There are other findings on the MRI that are highly suggestive of sarcoidosis. There are multicentric foci of LGE in linear and nodular form in the mid myocardial and subepicardial layers and likely in the pericardium and myocardium of the right ventricle.
There was associated global hypokinesis of the LV with an EF of 40%, and increase in the LV mass of 120 gms/ sq m
Ashley Davidoff MD
Key Issues  for Sarcoidosis
  • Similar to distribution in the lungs
    • Along lymphatics
    • Lymphatics of the heart are subepicardial
      • LGE characteristically subepicardial
    • Also patchy, multifocal  nodular mid myocardial
    • most characteristic
      • free wall and
      • medial basal septum (conduction abnormalities)

25-30% cardiac involvement

Chloroquin related Cardiomyopathy
Biventricular Infiltration
  • Other Infiltrative Disease with Biventricular Infiltration and Thickening
      • hemochromatosis
      • Less commonly
        • Fabry disease,
        • Dannon disease, and
        • Friedreich’s ataxia.