Journal ArticleDOI
Detection of early cardiac dysfunction in patients with severe beta-thalassemia and chronic iron overload.
Martin B. Leon,Jeffrey S. Borer,Stephen L. Bacharach,Michael V. Green,Edward J. Benz,Patricia Griffith,Arthur W. Nienhuis +6 more
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In this article, the authors used radionuclide cineangiography to determine left ventricular ejection fraction during exercise in 24 patients with transfusion-dependent, congenital anemias, 21 of whom had severe beta thalassemia.Abstract:
To detect early left ventricular dysfunction, we used radionuclide cineangiography to determine left ventricular ejection fraction during exercise in 24 patients with transfusion-dependent, congenital anemias, 21 of whom had severe beta thalassemia. Ejection fraction at rest was normal in 21 patients (>45 per cent) and in all patients was 53±2 per cent (mean ±S.E.M.) — not significantly different from the value in normal subjects. However, ejection fraction during exercise was normal in only 11 patients (53±3 per cent in all patients, P<0.001 as compared with the normal value). All eight patients who had received fewer than 100 transfusions but only three of 16 (19 per cent, P<0.001) who had received 100 or more transfusions had normal responses during exercise. Whereas echocardiographic fractional shortening at rest was normal in 16 of 19 patients studied, eight patients with normal fractional shortening had abnormal ejection-fraction responses to exercise. Thus, radionuclide cineangiography dur...read more
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Iron-chelating therapy and the treatment of thalassemia
TL;DR: The toxicity of this agent mandates a careful evaluation of the balance between risk and benefit of deferiprone in patients with thalassemia, in most of whom long-term deferoxamine is safe and efficacious therapy.
Journal ArticleDOI
Iron and the sex difference in heart disease risk
TL;DR: The hypothesis offered is that the greater incidence of heart diseases in men and postmenopausal women compared with the incidence in premenopausalWomen is due to higher levels of stored iron in these two groups, and the heart diseases of affluence are rare among impoverished peoples who are often iron deficient.
Journal ArticleDOI
Comparing the Means of Several Groups
TL;DR: Investigators need to become better acquainted with statistical techniques for making multiple comparisons and use inappropriate statistical methods to analyze the differences between group means.
Journal ArticleDOI
Clinical consequences of acquired transfusional iron overload in adults.
Andrew I. Schafer,R. G. Cheron,Robert G. Dluhy,Barry J. Cooper,Ray E. Gleason,J S Soeldner,H F Bunn +6 more
TL;DR: It is concluded that widespread subclinical organ dysfunction can result from transfusional iron overload developing in adulthood, and the pattern of organ involvement resembles that encountered in idiopathic hemochromatosis.
Journal ArticleDOI
Cardiovascular Function and Treatment in β-Thalassemia Major: A Consensus Statement From the American Heart Association
Dudley J. Pennell,James E. Udelson,Andrew E. Arai,Biykem Bozkurt,Alan R. Cohen,Renzo Galanello,Timothy M. Hoffman,Michael S. Kiernan,Stamatios Lerakis,Antonio Piga,John B. Porter,John Walker,John C. Wood +12 more
TL;DR: In this article, the authors report an expert consensus on the diagnosis and treatment of cardiac dysfunction in β-thalassemia major (TM), which does not cover other hemoglobias.
References
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Journal ArticleDOI
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Jeffrey S. Borer,Stephen L. Bacharach,Michael V. Green,Kenneth M. Kent,S E Epstein,G.S. Johnston +5 more
TL;DR: In this paper, the authors developed a noninvasive real-time radionuclide cineangiographic procedure permitting continuous monitoring and analysis of left ventricular function during exercise.
Journal ArticleDOI
Asymmetric Septal Hypertrophy: Echocardiographic Identification of the Pathognomonic Anatomic Abnormality of IHSS
TL;DR: The findings indicate that the detection of ASH identifies the pathognomonic anatomic abnormality in IHSS and includes a wider spectrum of patients (i.e. those without obstruction as well as clinically normal family members of patients with IhSS), and suggests that a more appropriate term for this disease entity is asymmetric septal hypertrophy or ASH.
Journal ArticleDOI
Iron in the heart. Etiology and clinical significance
TL;DR: G grossly visible CID are always associated with cardiac dysfunction and usually chronic cardiac failure, and are usually more extensive in ventricular than in atrial myocardium.