TN 8 (11-12)
DI 23022.953 Fulminant Giant Cell Myocarditis
COMPASSIONATE ALLOWANCE INFORMATION
FULMINANT GIANT CELL MYOCARDITIS
Fulminant GCM; Fulminant Myocarditis; Fulminant Non-ischemic Dilated Cardiomyopathy
Giant Cell Myocarditis (GCM) is a rare, autoimmune, cardiovascular disorder that causes inflammation of the heart muscle, ventricular tachycardia (a rapid heartbeat that starts in the ventricles) and often progresses to heart failure. Fulminant Giant Cell Myocarditis is a form of GCM that occurs suddenly and without warning. The cause of this disease is unknown. Symptoms of fulminant GCM include fever and other signs of infection including headache, muscle aches, sore throat, diarrhea, rashes, joint pain or swelling.
DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING
Diagnostic testing: Endomyocardial biopsy, clinical laboratory tests, coronary angiography, cardiac magnetic resonance imaging, and biomarkers of cardiac injury.
Physical findings: People with fulminant GCM may have a medical history significant for autoimmune diseases such as collagen vascular disease, inflammatory bowel disease, diabetes mellitus, sarcoidosis, thyrotoxicosis, Wegener granulomatosis or Loeffler syndrome.
ONSET AND PROGRESSION
Fulminant GCM has a poor prognosis and frequently requires heart transplantation or immunosuppression for long term survival. This disease most often occurs in people over 50 years of age. Mortality is associated with heart failure and ventricular arrhythmia.
Immunosuppressive drug therapy is initially used to treat fulminant GCM. Damage caused by heart muscle destruction and eventual heart failure eventually requires heart transplantation.
SUGGESTED PROGRAMMATIC ASSESSMENT*
Suggested MER for evaluation:
Suggested Listings for Evaluation:
Listing level severity must be documented.
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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.