Heart failure in beta thalassemia: a 5-year follow-up study

Kremastinos, Dimitrios Th.; Tsetsos, George; Tsiapras, Dimitrios; Karavolias, George; Ladis, Vassilios; Kattamis, Christos

doi:10.1016/s0002-9343(01)00879-8

Cited by 123 publications

(130 citation statements)

References 22 publications

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“…In the early stage, patients are usually asymptomatic. Restrictive cardiomyopathy usually occurs before dilated cardiomyopathy (7), in accordance with diastolic dysfunction, which normally happens before systolic dysfunction and overt heart failure (8)(9)(10)(11)(12).…”

Section: Clinical Insightmentioning

confidence: 99%

“…Although systolic dysfunction becomes obvious during the late stage, decreased contractile function has been demonstrated during the early stage of the disease (14)(15)(16). Left-sided heart failure is clinically more common than right-sided heart failure (12). However, it has been shown that right ventricular dysfunction develops earlier in asymptomatic TM patients (9,17).…”

Section: Clinical Insightmentioning

confidence: 99%

“…In addition to congestive heart failure, another major cause of death in this group of patients is cardiac tachyarrhythmias, which may occur simultaneously with a failing heart, leading to sudden cardiac death (12). Kremastinos et al (12) reported that the incidence of sudden death was approximately 11.6% in TM patients with left ventricular failure, which accounted for approximately 18.5% of total cardiac deaths.…”

Section: Clinical Insightmentioning

confidence: 99%

“…Kremastinos et al (12) reported that the incidence of sudden death was approximately 11.6% in TM patients with left ventricular failure, which accounted for approximately 18.5% of total cardiac deaths. Similar to mechanical dysfunction, electrophysiological dysfunction varies with the stage of disease.…”

Section: Clinical Insightmentioning

confidence: 99%

“…Findings in the early stage are usually accidental, including bradycardia, ST-T changes, infrequent premature atrial or ventricular contractions, first-degree atrioventricular block and evidence of left ventricular hypertrophy (18,19). In the late stage, frequent premature atrial or ventricular contractions, short runs of supraventricular tachycardia, atrial flutter and fibrillation, ventricular tachycardia and second-degree or complete heart block (including intraventricular block) have been demonstrated (7,12,19). Among these late electrocardiogram (ECG) Patients with thalassemia major have inevitably suffered from complications of the disease, due to iron overload.…”

Section: Clinical Insightmentioning

confidence: 99%

See 4 more Smart Citations

Iron overload thalassemic cardiomyopathy: Iron status assessment and mechanisms of mechanical and electrical disturbance due to iron toxicity

Lekawanvijit

Chattipakorn

2009

Canadian Journal of Cardiology

106

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Section: Clinical Insightmentioning

confidence: 99%

Section: Clinical Insightmentioning

confidence: 99%

Section: Clinical Insightmentioning

confidence: 99%

Section: Clinical Insightmentioning

confidence: 99%

Section: Clinical Insightmentioning

confidence: 99%

See 3 more Smart Citations

Iron overload thalassemic cardiomyopathy: Iron status assessment and mechanisms of mechanical and electrical disturbance due to iron toxicity

Lekawanvijit

Chattipakorn

2009

Canadian Journal of Cardiology

106

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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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The pathophysiological relationship and clinical significance of left atrial function and left ventricular diastolic dysfunction in β‐thalassemia major

et al. 2013

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Iron deposition in combination with inflammatory and immunogenetic factors is involved in the pathophysiology of cardiac dysfunction in b-thalassemia major. We investigated the mechanical and endocrine function of the left atrium and ventricle to identify early signs of dysfunction. We studied 90 patients (mean age: 29 6 11 years) with b-thalassemia and normal left ventricular function and 90 age and sex-matched healthy controls. Patients and controls underwent a thorough cardiac echocardiographic study and measurements of the b-type (NTproBNP) and atrial natriuretic peptides (proANP). Patients underwent 24-hr Holter recordings for arrhythmia monitoring. In the patient group, atria were affected early during the course of the disease, prior to diastolic and systolic left ventricular dysfunction. The E/E 0 ratio (E Doppler mitral fast inflow to the corresponding tissue Doppler E) continually increased with age (P < 0.05) and reached levels indicating left ventricular diastolic dysfunction (E/E 0 > 15) in the third decade whereas indexes of active and passive atrial function decreased gradually throughout life. In controls, the E/E 0 ratio continually increased with age but with later (fifth decade) appearance of diastolic dysfunction and a compensatory increase in atrial active function. Both natriuretic peptides were significantly increased in patients compared to controls (558 6 141 and 2,580 6 1,830 fmol/mL for NT-proBNP and proANP versus 332 6 106 and 1,331 6 1,134 fmol/mL, respectively). Atrial fibrillation was found in a subgroup of 23 (26%) patients, older in age with mild diastolic function and enlarged, depressed atria. In conclusion, atrial mechanical depression seems to be a very early sign of cardiac damage. It may become echocardiographically evident even before diastolic and systolic dysfunction and is associated to supraventricular arrhythmias.

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Heart failure in beta thalassemia: a 5-year follow-up study

Cited by 123 publications

References 22 publications

Iron overload thalassemic cardiomyopathy: Iron status assessment and mechanisms of mechanical and electrical disturbance due to iron toxicity

Iron overload thalassemic cardiomyopathy: Iron status assessment and mechanisms of mechanical and electrical disturbance due to iron toxicity

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

The pathophysiological relationship and clinical significance of left atrial function and left ventricular diastolic dysfunction in β‐thalassemia major

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