Left ventricular remodelling, and systolic and diastolic function in young adults with β thalassaemia major: a Doppler echocardiographic assessment and correlation with haematological data

G Bosi; R Crepaz; M R Gamberini; M Fortini; S Scarcia; E Bonsante; W Pitscheider; M Vaccari

doi:10.1136/heart.89.7.762

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Cardiovascular medicine

Left ventricular remodelling, and systolic and diastolic function in young adults with β thalassaemia major: a Doppler echocardiographic assessment and correlation with haematological data

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Response to Cheung
Bosi Giuliano
Published on: 23 November 2015
Arterial dysfunction contributes to 'beta-thalassaemia cardiomyopathy'
YF Cheung
Published on: 23 November 2015

Published on: 23 November 2015
Response to Cheung
- Bosi Giuliano, Associate Professor of Pediatric Cardiology
Dear Editor
We thank Dr Cheung for the interesting observations [1]concerning our paper.[2]
They suggest - on the basis of their study,[3] that an arterial dysfunction (probably due to iron overload) could contribute to the pathogenesis of the so-called “Thalassemic cardiomyopathy” causing an increase of arterial stiffness and, consequently, an increase of the pulsatile pressure if the ventricular systoli...
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Dear Editor
We thank Dr Cheung for the interesting observations [1]concerning our paper.[2]
They suggest - on the basis of their study,[3] that an arterial dysfunction (probably due to iron overload) could contribute to the pathogenesis of the so-called “Thalassemic cardiomyopathy” causing an increase of arterial stiffness and, consequently, an increase of the pulsatile pressure if the ventricular systolic pump function remains within normal ranges.
We wish to underline that, even in the presence of an increased impedence of the proximal arterial bed, the systemic vascular resistance (totally considered) could be reduced in a high cardiac output state due to the significant chronic anemia. This seems confirmed in our patients, in whom the afterload was increased because of an unfavourable mass/volume ratio. Anyway, as suggested by Cheung et al, an increased pulsatile vascular load might play an additional role in increasing the afterload. We obviously agree with the multifactorial pathogenesis of this "cardiomyopathy", but we think that the iron overload play an important role. Unfortunately, in our group of patients with a quite preserved left ventricular systolic pump function, we have found only a weak correlation between ferritin levels and EF% of the left ventricle. Being aware that the conventional endocardial indexes may overestimate the myocardial fibre shortening (an effect proportional to wall thickness) we have calculated (data not published) stress/velocity indexes at midwall level demonstrating a reduced contractility in 13% of our patients. So, more detailed information on the myocardial function in thalassemic patients affected by dilated and hypertrophic ventricles are needed. Doppler tissue imaging with strain and strain/rate imaging at different stages of the disease could probably give more information.
References
(1) Cheung YF. Arterial dysfunction contributes to 'beta-thalassaemia cardiomyopathy' [electronic response to Bosi G et al. Left ventricular remodelling, and systolic and diastolic function in young adults with ß thalassaemia major: a Doppler echocardiographic assessment and correlation with haematological data] heartjnl.com 2003
(2) Bosi G, Crepaz R, Gamberini MR, Fortini M, Scarcia S, Bonsante E, Pitscheider W, Vaccari M. Left ventricular remodelling, and systolic and diastolic function in young adults with ß thalassaemia major: a Doppler echocardiographic assessment and correlation with haematological data. Heart 2003;89:762-766.
(3) Cheung YF, Chan GC, Ha SY. Arterial stiffness and endothelial function in patients with beta-thalassemia major. Circulation 2002 Nov 12;106(20):2561-6.
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Conflict of Interest:
None declared.
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Published on: 23 November 2015
Arterial dysfunction contributes to 'beta-thalassaemia cardiomyopathy'
- YF Cheung, Paediatric Cardiologist
Dear Editor
Bosi and colleagues [1] suggested that significant volume load of the heart as a result of chronic anaemia in young adults with beta thalassaemia major is the main culprit of the so-called 'beta thalassaemic cardiomyopathy'. Their findings, based on echocardiographic assessment of left ventricular function, were similar to those reported previously.[2,3] Nonetheless, the role of arterial dysfunction in th...
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Dear Editor
Bosi and colleagues [1] suggested that significant volume load of the heart as a result of chronic anaemia in young adults with beta thalassaemia major is the main culprit of the so-called 'beta thalassaemic cardiomyopathy'. Their findings, based on echocardiographic assessment of left ventricular function, were similar to those reported previously.[2,3] Nonetheless, the role of arterial dysfunction in the pathogenesis of myocardial contractile dysfunction has likewise been ignored.
The fact that both the end systolic meridional and circumferential wall stress were increased in their patients would suggest an elevated left ventricular afterload. The importance of optimal ventriculo-arterial interaction in enhancing mechanical efficiency of the heart cannot be overemphasized. Unexpectedly, the systemic vascular resistance, which reflects the static vascular load, was significantly lower in their patients than controls. Furthermore, the significance of the pulsatile vascular load has not been discussed.
We have previously shown that teenagers and young adults with beta- thalassaemia major have arterial endothelial dysfunction and increased arterial stiffness.[4] The increase in arterial stiffness, relating probably to structural alteration as a result of iron overload and endothelial dysfunction, and increased wave reflection as a result of a faster pulse wave velocity increase the pulsatile afterload. Interaction between left ventricular ejection and systemic arterial impedance may thus render less favorable as the impedance modulus may no longer be the least at where the flow harmonics are highest.[5]
Our previous findings support the view of a multi-factorial etiology of LV failure in patients with beta-thalassaemia major.[6] Apart from myocardial iron deposition, myocarditis and immunogenetic profile, arterial dysfunction is also contributory.
References
(1) Bosi G, Crepaz R, Gamberini MR, et al. Left ventricular remodelling, and systolic and diastolic function in young adults with b thalassaemia major: a Doppler echocardiographic assessment and correlation with haematological data. Heart 2003;89:762-766.
(2) Lewis SB, Rachmilewitz AE, Amitai N, et al. Left ventricular function in b-thalassemia and the effect of multiple transfusion. Am Heart J 1978;96:636-645.
(3) Kremastinos DT, Tsiapras DP, Tsetos GA, et al. Left ventricular diastolic Doppler characteristics in beta-thalassemia major. Circulation 1993;88:1127-1135.
(4) Cheung YF, Chan GCF, Ha SY. Arterial stiffness and endothelial function in patients with beta-thalassaemia major. Circulation 2002;106;2561-2566.
(5) Nichols WW, O'Rourke MF. Vascular impedance. In McDonalds's Blood Flow in Arteries: Theoretical, Experimental and Clinical Principles. 4th ed. London: Edward Arnold; 1998:54-97, 243-293.
(6) Jessup M, Manno CS. Diagnosis and management iron-induced heart disease in Cooley's anemia. Ann N Y Acad Sci 1998;850:242-250.
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Conflict of Interest:
None declared.
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