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.