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Advanced Approach to Cardiac Amyloidosis

Modern diagnostic algorithms and targeted therapies for ATTR and AL cardiac amyloidosis.

Written by: Saygı Hospital Health Guide Editorial Board
Published:

This content is for general information; please consult your physician for diagnosis and treatment.

References (5)

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 Advanced Approach to Cardiac Amyloidosis?

Cardiac amyloidosis is an infiltrative cardiomyopathy caused by the deposition of abnormal protein in the myocardium. The two main forms are AL (light-chain) amyloidosis and ATTR amyloidosis (variant and wild-type).

An advanced approach is based on characteristic echocardiographic findings (concentric hypertrophy, apical sparing), cardiac MRI (late gadolinium enhancement), DPD/PYP bone scintigraphy for ATTR, and hematologic workup to distinguish AL.

Treatment for AL includes plasma-cell directed chemotherapy (bortezomib-based) and autologous stem cell transplantation. For ATTR, TTR stabilizers and RNA-silencing agents such as tafamidis, patisiran and inotersen are used.

Symptoms

Fatigue and exercise intolerance
Leg edema
Orthopnea
Carpal tunnel syndrome
Orthostatic hypotension
Palpitations and syncope
Macroglossia in AL type

Risk Factors

Advanced age
Family history of ATTR mutation
Monoclonal gammopathy for AL
Unexplained heart failure
Autonomic neuropathy
Bilateral carpal tunnel syndrome
Proteinuria and renal impairment

When to See a Doctor?

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

  • Unexplained left ventricular hypertrophy
  • Mismatch between low-voltage ECG and LVH
  • Refractory heart failure
  • Bilateral carpal tunnel syndrome
  • Orthostatic hypotension
  • Monoclonal protein findings

Treatment Methods

01
DPD/PYP scintigraphy and cardiac MRI
02
Hematologic workup to rule in AL
03
AL: bortezomib-based chemotherapy, ASCT
04
ATTR: tafamidis, patisiran, inotersen
05
Careful diuretic titration
06
Arrhythmia management
07
Multidisciplinary amyloidosis center

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.