Non cardiopatic and cardiopatic beta thalassemic patients: quantitative and qualitative cardiac iron deposition evaluation with MRI.
OBJECTIVE
Cardiomyopathy is one of the major complications of b thalassaemia major as a result of transfusional iron overload. The aim of our study is to evaluate with MR if there is any difference of iron deposition signal intensity (SI) or distribution between non-cardiopathic and cardiopathic thalassaemic patients in order to establish if there is a relationship between cardiopathy and iron deposition.


METHODS
We studied 20 patients affected by b thalassaemia major, of whom 10 cardiopathic and 10 non-cardiopathic, and 10 healthy volunteers as control group. Serum ferritin and left ventricular ejection fraction were calculated in thalassaemic patients. All patients were examined using a 1.5 MR unit with ECG-gated GE cine-MR T2*-weighted, SE T1-weighted and GE T2*-weighted sequences. In all cases, using an adequate ROI, the myocardial and skeletal muscle signal intensity (SI), the myocardial/skeletal muscle signal intensity ratio (SIR) and the SI average of the myocardium and skeletal muscle were calculated for every study group. The qualitative evaluation of iron deposition distribution was independently performed by three radiologists who analyzed the extension, the site and the morphology of iron deposition on the MR images and reported their observations on the basis of a four-level rating scale: 0 (absent), 1 (limited), 2 (partial), 3 (widespread deposition). The result of quantitative and qualitative evaluations were analysed with statistical tests.


RESULTS
Cardiac iron deposition was found in 8/10 non-cardiopathic thalassaemic patients and in all cardiopathic thalassaemic patients. We noticed a significant SI difference (p>0.05) between the healthy volunteer control group and the thalassaemic patients with iron deposition, but no significant SI difference in iron deposition between non-cardiopathic and cardiopathic thalassaemic patients in the areas evaluated. The qualitative evaluation revealed a different distribution of iron deposition between the two thalassaemic groups, with more widespread distribution in cardiopathic patients.


CONCLUSIONS
We found cardiac iron deposition also in non-cardiopathic b thalassaemic patients and a qualitative difference in cardiac iron distribution between non-cardiopathic and cardiopathic patients. The qualitative evaluation of cardiac iron deposition was useful for an easier classification of the disease, bypassing the SI quantitative value which is affected by the extremely uneven distribution of iron deposition and by the sampling technique used. MR evaluation of non-cardiopathic thalassaemic patients may be useful to evaluate early iron deposition and to establish the most suitable chelation therapy.
Macarini
L
L
DIMIMP, Sezione di Diagnostica per Immagini, Cattedra di Ematologia II, Università degli Studi, Bari, Italy. l.macarini@radiologia.uniba.it
Marini
S
S
Pietrapertosa
A
A
Scardapane
A
A
Ettorre
G C
GC
D000328:Adult; D009202:Cardiomyopathies / Q000175:diagnosis*; D005260:Female; D006801:Humans; D007501:Iron / Q000032:analysis*; D008279:Magnetic Resonance Imaging; D008297:Male; D018482:Muscle, Skeletal / Q000737:chemistry; D009206:Myocardium / Q000737:chemistry*; D013318:Stroke Volume; D017086:beta-Thalassemia / Q000150:complications* / Q000378:metabolism
D007501:Iron