The pathophysiological relationship and clinical significance of left atrial function and left ventricular diastolic dysfunction in [beta]-thalassemia major

Iron deposition in combination with inflammatory and immunogenetic factors is involved in the pathophysiology of cardiac dysfunction in [beta]-thalassemia major. We investigated the mechanical and endocrine function of the left atrium and ventricle to identify early signs of dysfunction. We studied...

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Published in:American journal of hematology 2014-01, Vol.89 (1), p.13
Main Authors: Kostopoulou, Anna G, Tsiapras, Dimitrios P, Chaidaroglou, Antigoni S, giannis, Dimitrios E, Farmakis, Dimitrios, Kremastinos, Dimitrios Th
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Hematology
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container_title American journal of hematology
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creator Kostopoulou, Anna G
Tsiapras, Dimitrios P
Chaidaroglou, Antigoni S
giannis, Dimitrios E
Farmakis, Dimitrios
Kremastinos, Dimitrios Th
description Iron deposition in combination with inflammatory and immunogenetic factors is involved in the pathophysiology of cardiac dysfunction in [beta]-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 [beta]-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
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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 [beta]-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&lt;0.05) and reached levels indicating left ventricular diastolic dysfunction (E/E'&gt;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. Am. J. Hematol. 89:13-18, 2014. © 2013 Wiley Periodicals, Inc. 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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. Am. J. Hematol. 89:13-18, 2014. © 2013 Wiley Periodicals, Inc. 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title The pathophysiological relationship and clinical significance of left atrial function and left ventricular diastolic dysfunction in [beta]-thalassemia major
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