Refractory biventricular heart failure in secondary hemochromatosis

Cardiac hemochromatosis usually presents with clinical congestive heart failure and an enlarged heart. A woman with secondary hemochromatosis and features of both restrictive and congestive cardiomyopathy with a normal sized heart is described. Despite all interventions she had progressive cardiac impairment and expired eight days following presentation. The clinical and laboratory findings of 49 cases of cardiac hemochromatosis during the last 50 years are reviewed. While the disease usually progresses slowly, a significant minority die within fourteen days of recognition. Not infrequently pericardial tamponade is entertained as a cause for this rapid deterioration; however, both a restrictive and congestive cardiomyopathy appear to be responsible for this catastrophic presentation.