Observations on the cardiovascular involvement in Friedreich's ataxia

TN James, C Fisch - American Heart Journal, 1963 - Elsevier
TN James, C Fisch
American Heart Journal, 1963Elsevier
The case of a young man with Friedrich's ataxia who died of intractable atrial arrhythmias
and cardiac failure is presented. At necropsy there was widespread involvement of the small
arteries of the myocardium (including the cardiac conduction system) and lungs. After critical
review of previous necropsy studies in this disease, it is suggested that the most likely
explanation for the cardiopathy of Friedreich's ataxia is obliteration of the small coronary
arteries. Potential significance of the pulmonary arteriopathy is briefly discussed.
Abstract
The case of a young man with Friedrich's ataxia who died of intractable atrial arrhythmias and cardiac failure is presented. At necropsy there was widespread involvement of the small arteries of the myocardium (including the cardiac conduction system) and lungs. After critical review of previous necropsy studies in this disease, it is suggested that the most likely explanation for the cardiopathy of Friedreich's ataxia is obliteration of the small coronary arteries. Potential significance of the pulmonary arteriopathy is briefly discussed.
Elsevier