FULMINANT GIANT CELL MYOCARDITIS

Fulminant GCM; Fulminant Myocarditis; Fulminant Non-Ischemic Dilated Cardiomyopathy

DESCRIPTION

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.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM CODING

Diagnostic testing:

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  Endomyocardial biopsy;

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  Clinical laboratory tests;

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  Coronary angiography;

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  Cardiac magnetic resonance imaging (MRI); and

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  Biomarkers of cardiac injury.

Physical findings: Individuals with fulminant GCM may have a medical history significant for autoimmune diseases such as:

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  Collagen vascular disease;

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  Inflammatory bowel disease;

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  Diabetes mellitus;

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  Sarcoidosis;

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  Thyrotoxicosis;

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  Wegener granulomatosis; or

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  Loeffler syndrome.

Symptoms of fulminant GCM include fever and other signs of infection including:

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  Headache;

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  Muscle aches;

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  Sore throat;

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  Diarrhea;

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  Rashes;

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  Joint pain; or

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  Swelling.

ICD-9: 422.91

ICD-10: I42.8

PROGRESSION

Fulminant GCM has a poor prognosis and frequently requires heart transplantation or immunosuppression for long term survival. This disease most often occurs in individuals over 50 years of age. Mortality is associated with heart failure and ventricular arrhythmia.

TREATMENT

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:

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  Clinical history and examination that describes the diagnostic features of the impairment;

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  Cardiac MRI report; and

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  Cardiac biopsy report.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets

4.02

Listing level severity must be documented.

4.05

Equals