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Cardiac Amyloidosis Diagnostic Algorithm

Differential diagnosis and treatment approaches for AL and ATTR cardiac amyloidosis.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Kardiyoloji department. Book Appointment →

What is Cardiac Amyloidosis Diagnostic Algorithm?

Cardiac amyloidosis is an infiltrative cardiomyopathy resulting from deposition of misfolded proteins in the myocardium. There are two main types: AL (light chain) amyloidosis and ATTR (transthyretin) amyloidosis.

The ATTR form may be hereditary (hATTR — TTR gene mutation) or acquired (wt-ATTR — age-related). Tc99m-PYP bone scintigraphy is a 99% specific non-invasive diagnostic method for ATTR. AL amyloidosis requires treatment of the underlying plasma cell dyscrasia.

Symptoms

Heart failure with preserved or mildly reduced EF (HFpEF)
Increased wall thickness (concentric hypertrophy-like)
Low-voltage ECG (disproportionate to wall thickness)
Carpal tunnel syndrome (bilateral — early sign in ATTR)
History of spinal stenosis (ATTR)
Orthostatic hypotension (autonomic neuropathy)

Risk Factors

Advanced age (>65 years — wt-ATTR)
TTR gene mutation (Val122Ile — 3-4% in African American population)
Plasma cell dyscrasia (AL)
Family history of amyloidosis
Unexplained HFpEF and left ventricular hypertrophy

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Unexplained increase in wall thickness on echocardiography
  • Discordance between low voltage ECG and hypertrophy
  • Inadequate response to HFpEF therapy
  • Heart failure with history of bilateral carpal tunnel syndrome

Treatment Methods

01
Tc99m-PYP scintigraphy (ATTR diagnosis — Grade 2-3 positivity)
02
Serum and urine free light chain analysis (rule out AL)
03
Tafamidis (TTR stabilizer — FDA approved in ATTR-CM)
04
Patisiran/inotersen (TTR silencer in hereditary ATTR)
05
Chemotherapy in AL amyloidosis (bortezomib, daratumumab)
06
Diuretic and supportive heart failure therapy

Which Department to Visit?

You can visit our Kardiyoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.