Early initiation of venesection is crucial for heart damage treatment in patients with HFE-related hereditary hemochromatosis
M Swiatczak, K Mlodzinski, A Swiatczak, K Sikorska, D Kaufmann, L Danilowicz SzymanowiczAbstract
Background
HFE-hemochromatosis (HH) causes iron-mediated organ injury, including the heart. We aimed to assess the impact of 3-year venesections on heart function using standard and advanced echocardiography.
Methods
Fifty-nine HH patients at different disease stages, and 29 matched healthy controls, were assessed at baseline and after 3 years of venesections. In addition to clinical evaluation, standard and advanced (speckle-tracking) echocardiography (STE) were performed in the participants.
Results
At baseline, standard echocardiography was within normal limits, but STE showed reduced global longitudinal strain (GLS) and lower rotational indices (apical and basal rotation, torsion and twist of the left ventricle) than in controls. After three years of venesections, newly diagnosed (≤6 months) patients showed significant improvement in rotation indices and left ventricular ejection fraction, whereas the medium- (6–24 months) and long-standing (>24 months) subgroups showed no meaningful recovery. GLS, which was significantly reduced compared with healthy controls at baseline (-21.0% [-22.1 – -19.3] vs -18.9% [-20.3 - -17.6]), p = 0.002), remained unchanged over the treatment in all subgroups.
Conclusions
In patients with HH, early initiation of venesection is crucial for improving heart function, but only regarding rotational, not longitudinal, indices, with particular attention to STE as the main diagnostic tool.