Cardiac Amyloidosis

Synonyms: Cardiac amyloidosis, cardiac AL, cardiac ATTR

Basic overview

Amyloidosis is the name given to a group of rare disorders in which amyloid deposits, consisting of misfolded proteins deposit and accumulate in tissues and organs of the body. As these deposits progressively build up they interfere with the working of the organs. Without treatment this will eventually leads to organ failure.

Symptoms

There are several types that are seen in clinical practice:

    • Wild-type transthyretin (ATTR)
      • Common type that predominantly affects the older population (age >60) with a strong male predominance
      • Heart involvement is by far the predominant organ involvement¬†
      • Often preceded by carpal tunnel syndrome in a large number of patients (~3-5 years prior to the diagnosis)
    • Hereditary transthyretin (variant ATTR)
      • Much less common than wild-type
      • Occurs when a gene mutation is inherited, leading to a life-long production of an abnormal transthyretin protein
      • Symptoms are dependent on the type of mutation but can vary between predominantly neurological, predominantly cardiac, or a mix of both
    • Light chain (AL)
      • Occurs due to misfolded immunoglobulin light chains that are produced by plasma cells in the bone marrow
      • Usually these plasma cells are benign, but occasionally the amyloid forming light chain protein can be produced by a cancer of plasma cells called Multiple Myeloma
      • About 15% of patients with Multiple Myeloma will also develop AL amyloidosis
    • AA amyloid
      • Clinically significant heart involvement is rare
      • Occurs with a prolonged and substantial increase the Serum Amyloid A (SAA) protein (released by the body during states of inflammation
      • Seen in the context of a long-term inflammatory disorder such as rheumatoid arthritis

Treatment

Treatment is very different for each type of amyloidosis and is always geared to the needs and health of the individual patient.

Treatment should ideally be recommended after careful review by experienced amyloidosis treatment teams.

Our Locations

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Cardiology Tasmania has 5 specialist centres located in Derwent Park, Huonville, Oatland, Swansea and Rokeby. As well as our fixed locations, our cardiology specialists and consultants travel to regional and remote areas to ensure all patients have access to cardiology services regardless of where they live.
Call Us:
0439 195 660
Derwent Park
  • Northcare Health Centre, 254 Main Road, Derwent Park, TAS

  • 62728869
Huonville
  • Huon Valley Health Centre, 85 Main Road, Huonville, TAS
  • 62642800
Oatlands
  • Midlands Multipurpose Health, 13 Church Street, Oatlands, TAS
  • 61350540
Swansea
  • Swansea General Practice: 37 Wellington Street, Swansea, TAS
  • 62578205
Rokeby
  • Knopwood Medical Centre: 46 South Arm Road, Rokeby, TAS
  • 62471019

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