TN 5 (07-11)
DI 23022.580 Primary Cardiac Amyloidosis
COMPASSIONATE ALLOWANCE INFORMATION
PRIMARY CARDIAC AMYLOIDOSIS
Stiff Heart Syndrome; Amyloidosis- Cardiac; Primary Cardiac Amyloidosis – AL type
Primary Cardiac Amyloidosis is a rare heart disease caused by deposits of abnormal protein (amyloid) in the heart tissue. These proteins slowly replace normal heart tissue causing the heart to become stiff. Individuals with this disease often experience difficulties with the way in which electrical signals move through the heart conduction system. These electrical disturbances can lead to arrhythmias and heart block. Heart function may be reduced. Cardiac amyloidosis is more common in men than in women. The disease is rare in people under age 40. Symptoms and signs may be similar to what is seen in congestive heart failure and may include excessive fluid retention, excessive urination at night, fatigue, heart palpitations, shortness of breath with activity, swelling of the legs and ankles, and difficulty breathing while lying down.
DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING
Chest or abdomen CT scan, coronary angiography, echocardiogram, MRI, and nuclear heart scans help diagnosis cardiac amyloidosis. A cardiac biopsy may confirm the diagnosis.
ICD 9: 277.3
ONSET AND PROGRESSION
Primary cardiac amyloidosis is a chronic condition that slowly gets worse. On average, persons with primary cardiac amyloidosis live less than 1 year.
Individuals with this disease are advised by their doctors to limit their intake of salt and fluids. Some patients are prescribed diuretics (water pills) to help the body remove excess fluid. Medications such as digoxin, calcium channel blockers and beta-blockers may be prescribed for individuals with atrial fibrillation (very rapid uncoordinated contractions of the heart). Other treatments may include chemotherapy, implantable cardioverter-defibrilator (AICD), pacemaker if there are problems with conduction (heart signals), and immunosuppressive agents. A heart transplant may be considered for individuals with very poor heart function.
SUGGESTED PROGRAMMATIC ASSESSMENT*
Suggested MER for Evaluation:
Clinical examination that describes diagnostic features of the disease and laboratory studies confirm the diagnosis.
Suggested Listings for Evaluation:
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* Adjudicators may, at their discretion, use the Medical Evidence of Record or Listings suggested to evaluate the claim. However, the decision to allow or deny the claim rests with the adjudicator.