Circulation, Vol 87, 748-754, Copyright © 1993 by American Heart Association
F Lattanzi, P Bellotti, E Picano, F Chiarella, A Mazzarisi, C Melevendi, G Forni, L Landini, A Distante and C Vecchio
BACKGROUND. Patients with beta-thalassemia major present with severe anemia
and need continuous transfusion therapy. The consequent iron overload leads
to hemochromatosis. Initial cardiac dysfunction has been documented even in
thalassemics without clinical manifestations of heart failure as well as by
conventional echocardiographic-Doppler techniques. The purpose of this
study was to assess the acoustic quantitative properties of myocardium in
patients with iron overload. METHODS AND RESULTS. Thirty-eight patients
with beta-thalassemia major, without clinical signs of cardiac failure, and
20 age- and sex-matched young controls were studied by echocardiography. An
on-line analysis of the ultrasonic radiofrequency signal was performed to
obtain quantitative operator-independent measurements of the integrated
backscatter (IB) signal of the ventricular septum and the posterior wall.
The integrated values of the radiofrequency signal were normalized for the
pericardial interface and expressed in percent (IB%). Thalassemic patients
had been receiving transfusion therapy for 16 +/- 5 years and had received
313 +/- 138 transfusion units; they all had received chelation treatment
(desferroxiamine) for 9 +/- 2 years. Patients and controls showed
comparable values of echocardiographically assessed percent fractional
shortening (32 +/- 3% versus 36 +/- 4%, p = NS), whereas thalassemics
showed higher values of left ventricular mass index (118 +/- 30 versus 98
+/- 15 g/m2, p < 0.05). The IB% values were higher in patients with
thalassemia major than in controls for both septum (35 +/- 14% versus 21
+/- 6%, p < 0.001) and posterior wall (16 +/- 6% versus 11 +/- 3%, p
< 0.001). In thalassemic patients, no significant correlation was found
between the septum IB% value and hematological parameters, such as the
total number of transfusions (r = 0.2, p = NS) or the mean ferritin value
(r = 0.1, p = NS). No significant correlation was also found between the
septum IB% value and the echocardiographically assessed left ventricular
mass index (r = 0.2, p = NS). CONCLUSIONS. These data demonstrate that
myocardial reflectivity is abnormally increased in patients with
thalassemia major under transfusion treatment, probably due to myocardial
iron deposits and/or secondary structural changes. These quantitatively
assessed abnormalities in regional reflectivity can be detected when
conventional echocardiographic parameters of systolic left ventricular
function are undistinguishable from normal controls.
ARTICLES
Quantitative ultrasonic analysis of myocardium in patients with thalassemia major and iron overload
Institute of Clinical Physiology, Pisa, Italy.
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