Desferrioxamine to Improve Cardiac Function in Iron-Overloaded Patients With Thalassaemia Major

Marcus, Robert; Davies, SallyC.; Bantock, H. M.; Underwood, S. R.; Walton, S.; Huehns, E. R.

doi:10.1016/s0140-6736(84)90439-2

Cited by 97 publications

(39 citation statements)

References 7 publications

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“…Thereafter, subcutaneous treatment was reintroduced. Aggressive DFO treatment of 85 -200 mg/kg per day intravenously was reported to reverse established symptomatic myocardial disease in three of five patients (14). However, the grade of heart failure was less severe than in the present case, with LVEF of 39 -52% before treatment and improvement only after 1 year of DFO therapy.…”

Section: Discussioncontrasting

confidence: 60%

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

Erfurth

Holmer²,

Nilsson

et al. 2004

European Journal of Endocrinology

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A 21-year-old woman with b-thalassemia major (b-TM) and GH deficiency developed end-stage heart failure, New York Heart Association (NYHA) functional class IV, within 3 months after withdrawal of recombinant human growth hormone (GH). A myocardial biopsy excluded myocarditis and showed moderate iron deposit in the heart. Before her admission, intensified treatments with digoxin, angiotensin-converting enzyme inhibitor, diuretics and extra chelation therapy (desferrioxamine (DFO)) had not improved her progressive heart failure. At admission, GH was reinstituted together with intensified treatment of cardiac drugs and low doses of DFO, and her heart failure reversed. Four months later, NYHA functional class II was reached and within 1 year her cardiac function was normalised. We suggest that GH deficiency due to iron-induced damage to the hypothalamic -pituitary axis can contribute to heart failure in adult patients with b-TM. European Journal of Endocrinology 151 161-166

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Section: Discussioncontrasting

confidence: 60%

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

Erfurth

Holmer²,

Nilsson

et al. 2004

European Journal of Endocrinology

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“…Continuous intravenous DFX chelation in 4 of the 60 patients resulted in a marked increase in the uri nary iron excretion and hence in a better iron balance with a concomitant improvement in the LVEF in the 2 patients studied, and a return from atrial fibrillation to sinus rhythm in a third [11], This reversal of cardiac dysfunction with more intensive chelation has been well described previously [4,8,15].…”

Section: Discussionmentioning

confidence: 78%

“…Long-term survival of patients who have clinical manifestations of heart disease is usually poor [3] even though intensive chelation can save some of them [4], It is important, therefore, to detect preclinical changes in heart function in order to im prove their iron chelation therapy [5][6][7][8][9][10]. Radionu clide study has been found to be a sensitive method in detecting cardiac dysfunction both at rest and during exercise [8,9], We have used this method to study a group of 60 well-transfused patients with variable compliance with subcutaneous DFX therapy in order to assess the incidence and severity of cardiomyopa thy and to monitor change in ejection fraction after using more regular and intensive iron chelation in pa tients with initial severe abnormalities.…”

Section: Introductionmentioning

confidence: 99%

High Incidence of Cardiomyopathy in Beta-Thalassaemia Patients Receiving Regular Transfusion and Iron Chelation: Reversal by Intensified Chelation

et al. 1990

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Cardiac scintigraphy has been performed in 60 β-thalassaemia major patients aged 8-35 years who received regular blood transfusions and subcutaneous desferrioxamine (DFX) chelation. Fifty-seven showed no clinical, radiological or electrocardiographic evidence of heart disease and 3 had clinically apparent cardiac failure. Twenty-two patients (37%) showed severe cardiac functional impairment defined by a resting left ventricular ejection fraction (LVEF) < 45% and/or a drop of > 12% on stress, while 19 were normal and 19 had a mild abnormality. There was no significant correlation between abnormality of LVEF and age, serum ferritin, number of units transfused, dose and duration of subcutaneous DFX therapy, liver disease or sexual maturation. Non-compliant patients (defined as the use of subcutaneous DFX less than 4 times weekly) generally showed worse cardiac function. Repeat study on 17 patients after 6-28 months of better compliance with subcutaneous or intravenous DFX (using an indwelling catheter) showed a significant overall improvement in LVEF associated with a significant drop in serum ferritin. We conclude that cardiac scintigraphy uncovers a high incidence of cardiac functional abnormality in asymptomatic, well-transfused thalassaemia patients, particularly those poorly compliant with chelation. Those with poor LVEF results should be offered intensive chelation therapy to improve cardiac function.

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“…However, heart failure is not prevented by intensification of iron chelation therapy. 4,5 It can thus be hypothesized that either deferoxamine is less effective than is believed or other factors might be involved in the pathogenesis of the heart failure.…”

mentioning

confidence: 99%

Heart Failure in β‐Thalassemia

Kremastinos

2001

Congestive Heart Failure

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Heart failure remains the main cause of death in ß-thalassemia despite the progress that has been made. Myocardial Heart failure remains the main cause of death in β-thalassemia, despite the progress that has been made by intensification of iron chelation therapy. 1 It has been traditionally considered the result of iron overload due to regular blood transfusions, intestinal iron hyperabsorption, and ineffective erythropoiesis during a patient's lifespan. 2,3 Regular, intense chelation therapy has improved quality of life and the survival rate by decreasing the secondary hemochromatosis. However, heart failure is not prevented by intensification of iron chelation therapy. 4,5 It can thus be hypothesized that either deferoxamine is less effective than is believed or other factors might be involved in the pathogenesis of the heart failure.Beta-thalassemia is not a pure iron storage disease and the pathophysiology of cardiac dysfunction is poorly understood and multifactorial in etiology. 6,7 Left-sided and subsequently biventricular heart failure appear early in the patients' life. This is the most common mode of heart failure in this disease, which is characterized at diagnosis by left ventricular systolic dysfunction, dilatation, and failure. 2,[8][9][10] Chronic myocardial iron deposition does not affect left ventricular relaxation but causes left ventricular myocardial restriction, with highly elevated pulmonary arteriolar resistance and pressure. It appears in the elderly β-thalassemia major population, who have the highest serum ferritin levels. These patients eventually develop right ventricular dilatation, while left ventricular dimensions and systolic function remain within the normal range. This is the pre-heart failure stage, with predominant symptoms and signs of right-sided heart failure. 11 In 1964, Engle et al. 8 first reported the coexistence of pericarditis and fatal arrhythmias with heart failure in β-thalassemia major. Of note, pericarditis usually coincides to some degree with myocarditis, an inflammatory heart disease that usually has an immunologic background. 2,12,13 Years later, our group 14 showed that acute infectious myocarditis in β-thalassemia major, in addition to causing acute failure, also caused chronic leftsided heart failure in 27.6% of myocarditis patients within approximately 3.5 years, compared to an ageand sex-matched β-thalassemic population with no difference in iron loading. The estimated prevalence of overt myocarditis in β-thalassemia was 4.5% in a population with clinical evidence of myopericarditis documented mainly by myocardial biopsy.

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Desferrioxamine to Improve Cardiac Function in Iron-Overloaded Patients With Thalassaemia Major

Cited by 97 publications

References 7 publications

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

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

High Incidence of Cardiomyopathy in Beta-Thalassaemia Patients Receiving Regular Transfusion and Iron Chelation: Reversal by Intensified Chelation

Heart Failure in β‐Thalassemia

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