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Cardiac amyloidosis



Heart, section through the middle
Heart, section through the middle
Dilated cardiomyopathy
Dilated cardiomyopathy


Cardiac amyloidosis

Alternative Names:

Amyloidosis - cardiac; Restrictive cardiomyopathy - amyloidosis; Primary cardiac amyloidosis - AL type; secondary cardiac amyloidosis - AA type; Stiff heart syndrome
Treatment:

Physical activity may continue as long as the patient can tolerate it. Diet restrictions vary with the extent of cardiomyopathy and heart failure. These may include salt and/or fluid restrictions.



Diuretics (water pills) may be given to remove excess fluid. Digoxin may be used cautiously to improve heart control in patients with atrial fibrillation. Daily weight measurement may be recommended. A weight gain of 3 or 4 pounds or more over 1 or 2 days can indicate excessive fluid accumulation.

Some patients benefit from chemotherapy or prednisone.

A pacemaker may be needed if the conduction system is involved. When heart function is very poor, a heart transplant may be considered for some patients (not in those with AL type amyloidosis, however, since their disease compromises many organs). In one type of secondary amyloidosis, liver transplantation is also required.


Expectations (prognosis):

Cardiac amyloidosis is a chronic and progressive condition. Your cardiologist may estimate your prognosis according to the thickness of your left ventricle and to the degree of restriction in your heart (cardiac stiffness). For primary amyloidosis, the median survival after diagnosis is about 2.1 years.


Complications:
  • congestive heart failure
  • atrial fibrillation or ventricular arrhythmias
  • sick sinus syndrome (occasionally)
  • symptomatic cardiac conduction system disease (arrhythmias related to abnormal conduction of impulses through the heart muscle)
  • low blood pressure and dizziness from excessive urination (from medication)
  • increased sensitivity to digoxin with the potential for digoxin toxicity and related arrhythmias
  • ascites (fluid accumulation in the abdomen)

Calling your health care provider:

Call your health care provider if you have this disorder and new symptoms develop, particularly:




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