Interstitial Fibrosis (Friedreich's ataxia)

There are many causes of interstitial fibrosis. It is seen in the presence of hypertrophy whether primary or secondary. This was a case of Friedreich’s ataxia, known to be associated with widespread interstitial fibrosis.

Friedreich’s ataxia (FA) is an autosomal recessive disorder which usually presents in childhood or adolescence with difficulty walking followed by muscular weakness and other neuromuscular manifestations. Cardiac involvement is frequent in FA. Fatalities are often due to heart failure. Symmetric hypertrophic cardiomyopathy is a frequent finding on ultrasound studies in young patients. Other cardaic manifestations include myocarditis and myocardial fibrosis as in this case. The conduction system can be involved with resultant tachyarrhythmias or bradyarrhythymias.

Contributed by Dr. Don B. Singer