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

Abstract：

:Iron deposition in combination with inflammatory and immunogenetic factors is involved in the pathophysiology of cardiac dysfunction in β-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 ± 11 years) with β-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 (NT-proBNP) 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'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' > 15) in the third decade whereas indexes of active and passive atrial function decreased gradually throughout life. In controls, the E/E' 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 ± 141 and 2,580 ± 1,830 fmol/mL for NT-proBNP and proANP versus 332 ± 106 and 1,331 ± 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.

journal_name

Am J Hematol

journal_title

American journal of hematology

authors

Kostopoulou AG,Tsiapras DP,Chaidaroglou AS,De Giannis DE,Farmakis D,Kremastinos DT

doi

10.1002/ajh.23581

subject

Has Abstract

pub_date

2014-01-01 00:00:00

pages

13-8

issue

eissn

0361-8609

issn

1096-8652

journal_volume

pub_type

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