Cardiac iron overload in thalassemic patients: An endomyocardial biopsy study

Abstract

Secondary heart failure induced by organ siderosis is the main cause of death in patients affected by thalassemia major. At present it cannot be predicted whether heart siderosis is correlated with iron overload and little is known about the real cardiac histological pattern of post transfusional hemochromatosis in patients with thalassemia major and intermedia. The study aim was to evaluate cardiac iron overload by non invasive and invasive techniques. Fifteen thalassemic patients were investigated and endomyocardial biopsy performed in ten revealed different grades of endomyocardial iron overload with histochemical positivity. Non invasive techniques are not able to furnish an exact picture of the cardiac hemochromatosis. There was a significant correlation between serum ferritin and myocardial iron grade. Patients with elevated ferritin levels and poor compliance to chelating therapy are at high risk of severe heart hemochromatosis. It was seen that endomyocardial biopsy is a useful tool in studying myocardial iron.

Is growth hormone deficiency contributing to heart failure in patients with beta-thalassemia major?

“…The serum ferritin levels were extremely high when she had manifest heart failure, although the levels remained high even when the heart failure was reversed (Table 1). This is in accordance with the rather unreliable time relation between heart failure and serum ferritin levels in these patients (13). Furthermore, extra DFO therapy shortly before admission did not improve the progressive heart failure.…”

“…The iron content in the biopsies was graded as moderate in the present case. An uneven distribution of iron can make quantification in biopsies unreliable, but a significant correlation between serum ferritin and myocardial iron grade in biopsies has been reported (13). Compared with matched controls, GH-deficient patients have shown an impaired left ventricular mass and LVEF (15 -18).…”

Evaluation of cardiac status in iron‐loaded thalassaemia patients following bone marrow transplantation: improvement in cardiac function during reduction in body iron burden

“…The distribution of iron within the heart is inhomogenous and cardiac biopsies may provide a misleading estimate of the degree of cardiac iron loading (Olson et al, 1987;Valdes-Cruz et al, 1982). Although a recent study (Lombardo et al, 1995) reported a modest correlation between the serum ferritin The horizontal line at a concentration of 7 mg iron/g liver, dry weight, indicates the upper limit of the 'optimal' range in patients with transfusional iron overload (Olivieri & Brittenham, 1997). The other horizontal line at a concentration of 15 mg iron/g liver, dry weight, indicates a 'threshold' for the development of cardiac disease and early death in patients with thalassaemia major and transfusional iron overload (Brittenham et al, 1994). concentration and myocardial iron, other studies (Barosi et al, 1989) have not observed this correlation.…”

Cardiac complications in beta-thalassemia: From mice to men

“…22,23 Moreover, it has been found upon autopsy that some small subgroups of older TI patients had developed myocardial siderosis. [24][25][26] Damage to cardiac tissues may have resulted from exposure to NTBI, without the accumulation of toxic iron species within myocytes. 27,28 This suggests that even without evidence of cardiac siderosis, TI patients may still be at risk of iron-related cardiac dysfunction.…”