Reversal of end-stage heart failure in juvenile hemochromatosis with iron chelation therapy: a case report.

ABSTRACT:

Background

Juvenile hemochromatosis is the most severe form of iron overloading phenotype. Although rare, it should be suspected in patients who present with hypogonadotropic hypogonadism, diabetes mellitus, or cardiomyopathy without a clear cause.

Case presentation

A young Serbian male presenting with end-stage heart failure was referred for extracorporeal membrane oxygenation. An endomyocardial biopsy revealed cytoplasmic iron deposits in myocytes. His condition was stabilized with biventricular assist devices and he was listed for heart transplantation. Iron chelation therapy was commenced and resulted in rapid removal of iron burden. Serial outpatient echocardiograms demonstrated myocardial recovery such that a successful biventricular assist device explant occurred 131

SUBMITTER: Cooray SD 

PROVIDER: S-EPMC5787235 | biostudies-literature | 2018 Jan

REPOSITORIES: biostudies-literature