71yo, thick walls, low voltage, and carpal tunnel release 8 yrs ago

71yo Black man, HFpEF, back in w/ volume overload again.

septum 16 mm, posterior wall 15 mm
LVEF 52%
QRS voltage low for that much wall
GLS -9% w/ apical sparing
bilateral carpal tunnel release 8 yrs ago

whats ur next test?

A. Endomyocardial biopsy
B. 99mTc-PYP scintigraphy, w/ serum and urine light chains at the same time
C. Cardiac MRI w/ gadolinium
D. Genetic testing for MYBPC3

Thick walls with low voltage is the pairing that moves amyloid to the front.
True hypertrophy adds myocyte mass and takes the voltage up with it.
Infiltration adds wall thickness made of material that carries no current, so
the voltage sits low against a ventricle that looks enormous. Apical sparing
on strain tells u the same story from a second direction.

The carpal tunnel is the part people read past. Amyloid lands in the
transverse carpal ligament years before the heart says anything, commonly 5
to 10 of them, and bilateral release in a man this age is a real clue rather
than a coincidence.

PYP earns it because it can make the diagnosis without a biopsy. The light
chains go off at the same time, because AL amyloid also takes up PYP, and
treating AL as ATTR sends that patient somewhere very bad.

so, where did u land? its B. And in a Black patient, V122I sits at the top of
the list, carried by 3 to 4% of Black Americans.

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