Beta-thalassemia is a hereditary anemia that is quite prevalent in Lebanon. Most patients with beta-Thalassemia are treated and followed up mostly at a multidisciplinary center, located in the suburban area of Beirut: the Chronic Care Center (CCC), operational since 1994. We will review the experience with beta-Thalassemia accumulated through this institution. Four hundred and twenty five patients, aged 2 to 68 years are followed up at the CCC. Sixty four percent have thalassemia major (TM) while 36% have thalassemia intermedia (TI). Lebanese patients with TM receive periodic packed red cell transfusions to maintain a pre-transfusional hemoglobin level of 10 gm/dl at all times and desferrioxamine is the standard iron chelator in use. Since 1994, 12 patients with TM have died from complications of their disease, with heart failure being responsible for the majority of deaths. The incidence of cardiac, endocrinologic, and infectious complications will be reviewed. Finally, both current and prospective preventive measures will be discussed, specifically educational campaigns and premarital screening. The effects of prevention are starting to show as the number of newly diagnosed disease is diminishing.

In patients with thalassaemia major and normal systolic function who have iron overload, the earliest sign of diastolic dysfunction is an impairment in left ventricular relaxation manifested as a prolonged isovolumic relaxation time.

SUMMARY The prevalence and clinical importance of false tendons were studied in 488 consecutive patients referred for echocardiography. Two hundred and eighty three (58%) patients had acquired heart disease, 91 (19%) had congenital heart disease, and 114 (23%) had normal hearts. Sixty six patients with normal hearts had innocent systolic murmurs and one had recurrent ventricular tachycardia. The overall prevalence of false tendons was 25% compared with 1 6% in a retrospective analysis of 763 cross sectional echocardiograms. When patients with innocent murmurs were excluded from statistical analysis, there was no significant difference in the prevalence of these tendons between children and adults, boys and girls, men and women, or between patients with acquired or congenital heart disease and normal patients. The prevalence of false tendons in patients with dilated left ventricles (57%), however, resembled that seen in necropsy studies. The prevalence of false tendons in patients with an innocent systolic murmur was 76% in children and 40% in adults, with an overall prevalence of 52%.False tendons are a common echocardiographic finding of no clinical importance except for their possible role in the genesis of innocent murmurs and ventricular arrhythmias. The echocardiographic detection of false tendons increases considerably when these structures are specifically sought and in conditions that result in left ventricular chamber dilatation.The echocardiographic features of left ventricular false tendons have been reported,`and the reliability of cross sectional echocardiography in the recognition of false tendons has been established.4 In a recent morphological study these left ventricular bands were found in 48% of children and 52% of adults.5 The reported prevalence of false tendons in patients referred for echocardiography, however, has varied widely between 0-4% to 61% (Table 1). We have re-evaluated the prevalence, location, and clinical importance of false tendons in patients referred for echocardiography and have attempted to explain the major discrepancies between morphological studies and most echocardiographic studies.

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Twelve thalassaemia major patients have been given deferiprone 75 mg/kg body weight daily as iron chelation therapy for 5 years. Their ages ranged from 18 to 34 years (mean 24.2) at the end of the study. Two patients were hepatitis C virus (HCV) mRNA positive and a further 5 were positive for HCV antibody. The mean serum ferritin level fell significantly from 4,302 ± 2,245 µg/l SD at baseline to 3,032 ± 1,155 µg/l at 2 years (p = 0.037) and 2,229 ± 1,070 µg/l (p = 0.007) at 5 years. At the end of the study, liver iron ranged from 3.59 to 23.7 mg/g dry weight (mean 11.9 ± 5.4), 3 patients having levels >15 mg/g. There was no significant change in serum AST levels, but ALT levels fell significantly at 2 years (p = 0.019) and 5 years (p = 0.001). Liver biopsy at the end of the study showed no evidence of hepatic fibrosis caused by deferiprone. Cardiac studies showed no overall change in left ventricular ejection fraction but a significant improvement in isovolumic relaxation time (p = 0.045). We conclude that in this albeit small group of thalassaemia major patients, deferiprone was a safe long-term method of iron chelation. In a minority, higher doses of deferiprone or a combination with desferrioxamine would be needed to lower liver iron below 15 mg/g.