By Amin H. Karim MD
A 72 year old dentist presented with several months of increasing shortness of breath. Echocrdiogram is as follows:
Coronary angiogram did not reveal any occlusive coronary disease.
Right ventricular endomyocardial biopsy showed a variable deposiiton of acellular material within the interstitium and replacing some myocardial fibres. Congo red stain and fluorescence microscopy with the Texas Red filter demonstrated strong positivity further supportive of amyloid deposition. The paraffin block was sent to Mayo Clinic for amyloid subtyping. it showed findings most consistent with age related amydoidosis since it did not detect amino acid sequence abnormality in the transthyretin protein.
November 2023: Four years after the above diagnosis patient is stable on treatment. He has a implantable automatic defibrillator and the current global ejection fraction is in the 45-50% range.
GlobeMedic 