Transthyretin amyloid cardiomyopathy is now treatable, so the cost of missing it has risen. It is worth suspecting in an older patient with heart failure, often with preserved ejection fraction, and unexplained thickening of the left ventricular wall.
Several clues raise the suspicion: an electrocardiogram whose voltages look low or normal despite a thick ventricle, a history of bilateral carpal tunnel syndrome or lumbar canal stenosis, poor tolerance of standard heart failure drugs because of low blood pressure, and aortic stenosis in an elderly patient with disproportionate symptoms.
The diagnostic path is established: screen for a monoclonal protein with serum free light chains and serum and urine immunofixation, then, if negative, use bone scintigraphy. This sequence confirms ATTR non-invasively in most cases and is worth knowing before referral.
- Suspect ATTR cardiomyopathy in older patients with heart failure and unexplained left ventricular thickening.
- Low or normal ECG voltages despite a thick ventricle is a classic discordance.
- Bilateral carpal tunnel syndrome and lumbar canal stenosis often precede the cardiac diagnosis by years.
- Exclude a monoclonal protein with free light chains and immunofixation before bone scintigraphy.
- A positive bone scan with no monoclonal protein confirms ATTR non-invasively in most cases.
Why it matters
ATTR cardiomyopathy has treatment now, so recognising the red flags changes outcomes rather than just the label.
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