Thalassemia Heart Disease

Aessopos, Athanasios; Farmakis, Dimitrios; Deftereos, Spyros; Tsironi, Maria; Tassiopoulos, Stergios; Moyssakis, Ioannis; Karagiorga, Markisia

doi:10.1378/chest.127.5.1523

Cited by 187 publications

(60 citation statements)

References 33 publications

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“…Cardiovascular involvement represents a leading cause of mortality and morbidity both in SCD and in β-thalassemia and has extensively been described in these two conditions [3,4]. Studies addressing cardiac status in patients with SCD have applied a wide range of diagnostic modalities, including electrocardiography, Doppler echocardiography, scintigraphic techniques, cardiac catheterization and angiography, and more recently cardiac magnetic resonance imaging (CMR) [3].…”

Section: Introductionmentioning

confidence: 99%

“…Studies addressing cardiac status in patients with SCD have applied a wide range of diagnostic modalities, including electrocardiography, Doppler echocardiography, scintigraphic techniques, cardiac catheterization and angiography, and more recently cardiac magnetic resonance imaging (CMR) [3]. In some of those studies, patient populations have included a number of S-Thal cases; most of them have focused on specific cardiovascular aspects, such as left ventricular (LV) function, valvular involvement, coronary artery involvement, endothelial function, or right heart function [4][5][6][7]. In the present study, motivated by some preliminary data showing that pulmonary hypertension (PHT), although an important determinant of prognosis in sickle cell anemia, was rather mild in S-Thal patients [5], we sought to perform a global evaluation of cardiac function in a large cohort of Greek S-Thal patients and discuss our findings in comparison with the existing data in sickling syndromes.…”

Section: Introductionmentioning

confidence: 99%

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Cardiac involvement in sickle β-thalassemia

et al. 2008

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Cardiovascular involvement is a leading cause of mortality and morbidity in patients with inherited hemoglobinopathies, but it has not been adequately assessed in sickle β-thalassemia. We evaluated 115 sickle β-thalassemia patients, aged 34±14 years, along with 50 healthy controls, by resting echocardiography. Patients with systolic left ventricular (LV) dysfunction or severe pulmonary hypertension (PHT) also underwent left and right cardiac catheterization and cardiac magnetic resonance imaging (CMR). Left and right chamber dimensions, LV mass, and cardiac index were significantly higher in patients compared to controls (p<0.001 in most cases). Three patients (2.9%) had reduced LV ejection fraction (<55%); mean LV ejection fraction was significantly lower in patients (p< 0.001). Left and right ventricular systolic tissue Doppler indices and LV diastolic tissue Doppler indices were also impaired in patients. All three patients with systolic LV dysfunction had normal coronary arteries and mild myocardial iron load (CMR T2* values, 18-25 ms). Systolic pulmonary artery pressure was significantly higher in patients compared to controls (p=0.002); PHT was present in 28 patients (27%), while severe PHT in three (2.9%). In three patients with severe PHT, only one had impaired LV ejection fraction and increased pulmonary wedge pressure. Overall, three patients (2.9%) had a history of heart failure, two with systolic LV dysfunction, and one with severe PHT. Cardiac involvement in sickle β-thalassemia concerns biventricular dilatation and dysfunction along with PHT, leading to congestive heart failure.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Cardiac involvement in sickle β-thalassemia

et al. 2008

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“…This differs from thalassemia intermedia in which left ventricular function is usually preserved (although pulmonary hypertension is a recognized complication) 8. In this case report, the patient's clinical condition rapidly deteriorated due to left ventricular failure as a result of noncompliance with chelation therapy.…”

Section: Discussionmentioning

confidence: 75%

Acute cardiac decompensation in a patient with beta‐thalassemia and diabetes mellitus following cessation of chelation therapy

Lioudaki

Whyte

2016

Clinical Case Reports

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Key Clinical MessagePatients with higher liver iron stores are likely to have a worse cardiac outcome following noncompliance with chelation. Cardiovascular magnetic resonance identifies myocardial siderosis allowing optimization of iron chelation regimes. Diabetes puts thalassemic patients at increased risk of myocardial fibrosis. Dual chelation therapy with deferoxamine and deferiprone offers improved cardiac outcomes.

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“…Endotelyal disfonksiyona bağlı doku hipoksisi, anemiye bağlı yüksek kardiyak output ve demir birikimine bağlı oksidatif doku hasarı nedeniyle pulmoner vasküler remodelling gibi çeşitli mekanizmalar öne sürülmüştür [5] . Gerek talasemi intermediada gerekse talasemi majörde eski çalışmalar pulmoner hipertansiyon sıklığının %65-70 arasın-da olduğu bildirse de [6,7] daha yeni çalışmalarda bu oranın %10'larda olduğu görülmektedir [8] . Her ne kadar bu orandaki düşüşün tedavideki gelişmeler ve hastaların yakın izlemi ile ilişkili olduğu söylense de henüz bu teoriyi destekleyen yeterli veri bulunmamaktadır [9] .…”

Section: Discussionunclassified