Myocarditis in β-Thalassemia Major

Kremastinos, Dimitrios Th.; Tiniakos, G.; Theodorakis, George; Katritsis, Demosthenes G.; Toutouzas, Pavlos

doi:10.1161/01.cir.91.1.66

Cited by 119 publications

(48 citation statements)

References 25 publications

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“…20 -22 More specifically, myocarditis was identified as a cause of LV failure, whereas the HLA-DQA1*0501 allele and the apolipoprotein E e4 allele were both associated with an increased prevalence of adverse LV remodeling and reduced LVEF in patients with thalassemia major. [23][24][25] Besides direct myocardial injury, iron overload may also affect the heart indirectly through its effects on other organs. Thus, hepatic dysfunction, endocrinopathies (diabetes mellitus, hypothyroidism, hypoparathyroidism), and immune deficiency resulting from iron overload may contribute to the pathophysiology of IOC.…”

Section: Pathogenetic Mechanismsmentioning

confidence: 99%

“…10 Those 2 phenotypes are followed by several other manifestations including conduction system abnormalities, tachyarrhythmias, and perimyocarditis. 1,23,28 In the early stages of the disease, myocardial iron overload is expressed as diastolic LV dysfunction. Spirito et al 4 studied 32 young thalassemia major patients with preserved LVEF; transmitral Doppler flow velocities revealed a restrictive LV filling pattern in 50% of those patients.…”

Section: Phenotypic Expressionmentioning

confidence: 99%

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Iron Overload Cardiomyopathy in Clinical Practice

2011

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Section: Pathogenetic Mechanismsmentioning

confidence: 99%

Section: Phenotypic Expressionmentioning

confidence: 99%

Iron Overload Cardiomyopathy in Clinical Practice

2011

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“…4 We have recently reported that apart from iron overload, myocarditis is another contributing factor in the pathogenesis of heart failure. 5 Myocarditis can cause acute or chronic left ventricular systolic dysfunction and dilatation, which appear to be mediated by predominantly immunologic mechanisms rather than viral infection and replication. 6,7 In this respect, apart from iron deposition itself, myocardial dysfunction might be related to a late autoimmune process in a manner similar to that observed in idiopathic dilated cardiomyopathy due to acute myocarditis.…”

mentioning

confidence: 99%

Association of Heart Failure in Homozygous β-Thalassemia With the Major Histocompatibility Complex

et al. 1999

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Background —In β-thalassemia major, heart failure primarily affecting left ventricular systolic function is the most common complication and cause of death. Apart from iron deposition, it has been recently reported that myocarditis might be another contributing factor in the pathogenesis of acute or chronic heart failure, acting possibly through an autoimmune mechanism. In an attempt to assess the role of immunogenetic factors in the development of heart failure associated with β-thalassemia major, we studied the frequency of major histocompatibility antigens/alleles A, B, DR, and DQ in homozygous β-thalassemic patients with and without heart failure primarily affecting the left ventricle. Methods and Results —Forty-five consecutive unrelated Greek patients with homozygous β-thalassemia and left-sided chronic heart failure were studied. Fifty-eight unrelated Greek patients with homozygous β-thalassemia without heart failure and 130 unrelated Greek healthy controls were also studied. In all subjects, class I HLA-A and -B typing was performed by the complement-mediated lymphocytotoxicity assay, whereas class II HLA-DR and -DQ typing was performed by polymerase chain reaction. HLA-DRB1*1401 allele frequency was significantly increased in patients with β-thalassemia major without left-sided heart failure compared with those with heart failure (corrected P [ P c ]=0.02, odds ratio 0.1) and healthy controls ( P c =0.001). HLA-DQA1*0501 allele frequency was increased in patients with heart failure compared with patients without heart failure ( P c =0.04, odds ratio 14) and healthy controls ( P c =0.004). Conclusions —Differences exist in the immunogenetic profile between homozygous β-thalassemic patients with and without left-sided heart failure, raising the possibility that genetically defined immune mechanisms may play an important role in the pathogenesis of heart failure in β-thalassemia.

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“…In thalassemia, iron overload results from both elevated gastrointestinal absorption and transfusion therapy [14]. In nontransfusion dependent thalassemia (NTDT), iron overload is primarily due to gut absorption.…”

Section: Discussionmentioning

confidence: 99%