EVIDENCE OF ABNORMAL LEFT VENTRICULAR FUNCTION IN PATIENTS WITH THALASSAEMIA MAJOR: AN ECHOCARDIOGRAPHY BASED STUDY

Authors

  • Muhammad Sohail Arshad
  • Syed Najam Hyder

Abstract

Background: Thalassaemia represent one of the most common single gene disorder causing a majorpublic health problem in Pakistan. Nearly 100,000 people are born worldwide with this severe blooddisorder every year. Over the last 3 decades, the development of regular transfusion therapy and ironchelation has dramatically improved the quality of life and transformed thalassaemia from a rapidlyfatal disease to a chronic disease compatible with prolonged survival. Objective of this observationalcross sectional study was to determine the effects of chronic anaemia and transfusional iron overload onthe left ventricular function using Doppler echocardiography. Methods: This study was conducted inthe Department of Paediatric Cardiology, The Children’s Hospital & Institute of Child Health, Lahore,Pakistan from 1st April 2006 to 30th September 2007. The study comprised of 50 consecutive cases ofbeta-Thalassaemia major and 30 controls with normal haemoglobin and electrophoresis pattern. β-Thalassaemia major patients were diagnosed on the basis of haemoglobin electrophoresis. Patients withany congenital or acquired heart disease, concurrent infective disorder and with history of cardiacsurgery were excluded from the study. 2-D, M-mode and Doppler echocardiography was performed inall the study cases and controls. Statistical comparison of study cases and controls was conducted byusing unpaired t-test. Results: The age of the patients ranged from 2 years to 25 years with mean age of9.65 years. Males were 34 (68%) and females were 16 (32%). None of the study cases was on regularchelation programme while 31 (62%) patients were on irregular chelation with single dose ofintravenous desferrioxamine only at the time of blood transfusion. 19 (38%) of the patients had LVdysfunction in the form of isolated systolic dysfunction in 2 (4%), isolated diastolic dysfunction in 15(30%) while global dysfunction in 2 (4%) of the patients. Left ventricular dimensions, stroke volumeand E/A ratio were found considerably high in the study group. Conclusion: A very high percentage ofThalassaemia patients have cardiac involvement as LV dysfunction. This is mainly due to chronicanaemia, poor compliance with chelation therapy and non-availability of proper cardiac monitoring.Regular assessment of cardiac function may help a lot to improve the quality of life of these patientsand may reduce the morbidity and mortality to a great extent.Keywords: Beta Thalassaemia major, Echocardiography, Left ventricular dysfunction

References

Ahmed S, Saleem M, Modell B, Petrou M. Screening

extended families for genetic hemoglobin disorders in

Pakistan. N Engl J Med 2002;347:1162–8.

Verma IC, Choudhry VP, Jain PK. Prevention of

Thalassemia: A Necessity in India. Indian J Pediatr

;59:649–54.

Aessopos A, Farmakis D, Hatziliami A, Fragodimitri C,

Karabtsos F, Joussef J, et al. Cardiac status in well treated

patients with Thalassemia Major. Eur J Haemat

;73:359–66.

Hoffband AV. A sensitive test for early myocardial ironloading. Eur Heart J 2003;24:26–7. Comment on: Eur Heart J

;24:113–9.

Chotivittayatarakorn P, Seksarl P, Pathmanand C,

Thisyakorn, Sueblinvong V. Cardiac dysfunction in betaThalassemic children. J Med Assoc Thai 1993;76:591–6.

Hahalis G, Alesopoulos D, Kremastinos DT, Zoumbos NC.

Heart failure in beta-thalassemia syndromes: a decade of

progress. Am J Med 2005;118:957–67.

Aessopos A, Kati M, Farmakis D. Heart diseases in

thalassemia intermedia: a review of underlying

pathophysiology. Haematologica 2007;92(5):658–65.

Aldouri MA, Wonk B, Hoffbrand AV, Flynn D/M, ward sE,

Agnew JE et al. High incidence of cardiomyopathy in β-

thalassemia patients receiving regular transfusion and iron

chelation: reversal by intensified chelation. Acta Haematol

;84:113–7.

O’leary PW, Durongpisitkul K, Cordes TM. Diastolic

ventricular function in children: A Doppler

echocardiographic study establishing normal values and

predictors of increased ventricular end diastolic pressure.

Mayo Clin Proc 1998;73:616–28.

Hahalis G, Manolis AS, Apostolopoulos D. Right ventricular

cardiomyopathy in beta-thalassemia major. Eur Heart J 2002;

:147–56. Comment in: Eur Heart J 2002;23:102–5.

Anderson LJ, Westwood MA, Holden S. Myocardial iron

clearance during reversal of siderotic cardiomyopathy with

intravenous desferrioxamine: a prospective study using T2

cardiovascular magnetic resonance. Br J Haematol

;127:348–55.

Davis BA, O’Sullivan C, Jarritt PH, Porter JB. Value of

sequential monitoring of left ventricular ejection fraction in

management of thalassemia major. Blood 2004;104:263–9.

McMahon CJ, Nagueh SF, Eapen RS . Echocardiographic

predictor of adverse clinical events in children with dilated

cardiomyopathy: a prospective clinical study. Heart

;90:908–15.

Bosi G, Crepaz R, Gamberini MR, Fortini M, Scarcia S,

Bonsante E, et al. Left ventricular remodeling, and systolic

and diastolic function in young adults with beta-thalassemia

major: a Doppler echocradiographic assessment and

correlation with hematological data. Heart 2003;89:762–6.

Aessopos A, Deftereos S, Tsironi M, Karabatsos F, Yousaf J,

Fragodimitri C, et al. Predictive echo-Doppler indices of

left ventricular impairment in B-thalassemic patients. Ann

Hematol 2007;86(6):429–34.

Kremastinos DT. Heart failure in beta-thalassemia major.

CHF 2001;7:312–4.

J Ayub Med Coll Abbottabad 2009;21(2)

http://www.ayubmed.edu.pk/JAMC/PAST/21-2/Sohail.pdf 41

Atiq M, Bana M, Ahmed US, Bano S, Yousuf M, Fadoo Z, et

al. Cardiac disease in beta-thalassemia major: is it reversible?

Singapore Med J 2006;47:693–6.

Spiritio P, Lupi G, Melevendi C, Vecchio C. Restrictive

diastolic abnormalities identified by Doppler

echocardiography in patients with thalassemia major.

Circulation 1990;82:88–94.

Kremastinos DT, Tsiapras DP, Tsetsos GA, Rentoukas EI,

Vretou HP, Toutouzas PK. Left ventricular diastolic Doppler

characteristics in Thalassemia Major. Circulation

;88:1127–35.

Favilli S, De simone L, Mori F, Pollini I, Cecchi, Zuppiroli A

et al. The Cardiac changes in thalassemia major: Their

assessment by Doppler echocardiography. G Ital Cardiol

;23:1195–200.

Taksande A, Vilhekar K, Chaturvedi P, Jain M, Bang A,

Ganvir B. Cardiac changes in beta-thalassemia major

children: assessment by echocardiography. J Mahatma

Gandhi Inst Medl Sci 2006;11(i):45–51.

Vaccari M, Crepaz R, Fortini M, Gamberini MR, Scaricia S,

Pitscheider W, et al. Left ventricular remodeling, systolic

function, and diastolic function in young adults with betathalassemia intermedia: a Doppler echocardiography study.

Chest 2002;121:506–12.

Downloads

Published

2009-06-01

Issue

Vol. 21 No. 2 (2009): JOURNAL OF AYUB MEDICAL COLLEGE, ABBOTTABAD

Section

ORIGINAL ARTICLE

License

Journal of Ayub Medical College, Abbottabad is an OPEN ACCESS JOURNAL which means that all content is FREELY available without charge to all users whether registered with the journal or not. The work published by J Ayub Med Coll Abbottabad is licensed and distributed under the creative commons License  CC BY ND Attribution-NoDerivs. Material printed in this journal is OPEN to access, and are FREE for use in academic and research work with proper citation. J Ayub Med Coll Abbottabad accepts only original material for publication with the understanding that except for abstracts, no part of the data has been published or will be submitted for publication elsewhere before appearing in J Ayub Med Coll Abbottabad. The Editorial Board of J Ayub Med Coll Abbottabad makes every effort to ensure the accuracy and authenticity of material printed in J Ayub Med Coll Abbottabad. However, conclusions and statements expressed are views of the authors and do not reflect the opinion/policy of J Ayub Med Coll Abbottabad or the Editorial Board.

USERS are allowed to read, download, copy, distribute, print, search, or link to the full texts of the articles, or use them for any other lawful purpose, without asking prior permission from the publisher or the author. This is in accordance with the BOAI definition of open access.

AUTHORS retain the rights of free downloading/unlimited e-print of full text and sharing/disseminating the article without any restriction, by any means including twitter, scholarly collaboration networks such as ResearchGate, Academia.eu, and social media sites such as Twitter, LinkedIn, Google Scholar and any other professional or academic networking site.