Myocardial infarction with normal coronary arteries in S/ thalassaemia patients

Aessopos, Athanasios; Samarcos, M.; Hadjinikolaou, P.; Vaiopoulos, George; Skoumas, V.; Kolainis, I.; Loukopoulos, D

doi:10.1093/oxfordjournals.eurheartj.a014851

Cited by 5 publications

(5 citation statements)

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“…Evaluation of myocardial iron by CMR in the three patients with impaired LV ejection fraction revealed absent or borderline iron load (T2* values of 18, 20, and 25 ms, respectively, Table 3). Furthermore, the heart may be directly affected by the main disease as the sickling process may induce myocardial ischemia and heart muscle injury, despite the presence of angiographically normal coronary arteries [6,31]. Actually, in one of our patients with reduced LV function, a previous myocardial scintigraphic perfusion scan was positive for stress-induced ischemia.…”

Section: Discussionmentioning

confidence: 80%

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: Discussionmentioning

confidence: 80%

Cardiac involvement in sickle β-thalassemia

et al. 2008

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“…About half of these patients have a positive stress test, but in catheterizations and post-mortem evaluations, reduced atherosclerosis has been shown [11]. Effort-induced ischemia with normal coronary arteries has been also shown recently in sickle-thalassemia patients [12].…”

Section: Discussionmentioning

confidence: 99%

Myocardial infarction in a patient with β‐thalassemia major: First report

Fridlender

Rund

2003

American J Hematol

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Thalassemia is the most common hereditary anemia throughout the world. Survival in its most severe long-term form, b-thalassemia major, has significantly risen in the last decades. Cardiac morbidity-heart failure and dysrhythmias-is still the most common cause of mortality in these patients. We describe herein a case of myocardial infarction with normal coronary arteries in a 48-year-old patient with b-thalassemia and no other recognized risk factors for coronary artery disease. Thromboembolic phenomena, a known situation in these patients, occur at a frequency of 4-5%. However, as far as we know, this is the first report in the literature of myocardial infarction in association with b-thalassemia. With the notable improvement in the life expectancy of thalassemia patients, ischemic heart disease may become an important complication encountered in these patients. Am.

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“…Following an initial observation of myocardial infarction in two SThal patients with patent coronary arteries, a subsequent study of radionuclide perfusion imaging in 30 SThal patients revealed stress‐induced perfusion defects in 27% of them, reversible in all but one case; coronary angiograms did not reveal significant lesions in any of those patients . A case of unstable angina in a TI patient with severe calcified and yet patent coronary arteries has also been reported …”

Section: Clinical Phenotypesmentioning

confidence: 97%

“…Myocardial ischaemia is a particular mechanism of LV dysfunction in patients with SCD . Ischaemia is not associated with the presence of typical CAD, as significant coronary artery lesions are not generally seen in those patients . It seems to result mainly from small‐vessel disease, in which the sickling process plays the key role.…”

Section: Pathophysiologymentioning

confidence: 99%

Heart failure in haemoglobinopathies: pathophysiology, clinical phenotypes, and management

Farmakis

Triposkiadis

Lekakis

et al. 2016

European J of Heart Fail

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Hereditary haemoglobinopathies, mainly beta-thalassemia and sickle cell disease, constitute the most common monogenic disorders in humans, and although once geographically confined, they are currently globally distributed. They are demanding clinical entities that require multidisciplinary medical management. Despite their genotypic and phenotypic heterogeneity, the haemoglobinopathies share several similarities in pathophysiology, clinical manifestations, therapeutic requirements, and complications, among which heart failure (HF) represents a leading cause of mortality and morbidity. However, haemoglobinopathies have generally been addressed in a rather fragmentary manner. A unifying approach focusing on the underlying similarities of HF attributes in the two main entities might contribute to their better understanding, characterization, and management. In the present review, we attempt such an approach to the pathophysiology, clinical phenotypes, and management of HF in haemoglobinopathies.

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Myocardial infarction with normal coronary arteries in S/ thalassaemia patients

Cited by 5 publications

References 0 publications

Cardiac involvement in sickle β-thalassemia

Cardiac involvement in sickle β-thalassemia

Myocardial infarction in a patient with β‐thalassemia major: First report

Heart failure in haemoglobinopathies: pathophysiology, clinical phenotypes, and management

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