Role of Left Atrial Structure and Function in the Early Prediction of Cardiac Iron Overload in Transfusion-Dependent β-Thalassemia Patients

Document Type : Original Article

Authors

1 Echocardiography Research Center, Rajaie Cardiovascular, Medical, and Research Center, Iran University of Medical Sciences, Tehran, IR Iran.

2 Department of Cardiology, Farshchian Heart Center, Hamadan University of Medical Sciences, Hamadan, IR Iran.

3 Department of Thalassemia Clinic, Transfusion Research Center, High Institute for Research and Education in Transfusion Medicine, Tehran, IR Iran.

4 Rajaie Cardiovascular, Medical, and Research Center, Iran University of Medical Sciences, Tehran, Department of Thalassemia Clinic,

5 Department of Cardiology, Khomeini Hospital, Jundishapour University of Medical Sciences, Ahvaz, IR Iran.

Abstract

Background: β-thalassemia is the most common monogenic disease caused by abnormalities in the synthesis of the β-chain of hemoglobin.
Methods: From January 2018 to September 2018, 90 patients (age >18 y) with β-thalassemia major or intermedia who referred to Rajaei Cardiovascular, Medical, and Research Center, Tehran, Iran, for the assessment of myocardial iron overload were enrolled. All the patients were receiving regular blood transfusions and chelating therapy. Comprehensive transthoracic echocardiographic studies consisting of 2D echocardiography, tissue Doppler imaging, and real-time 3D echocardiography were performed.
Results: A total of 90 patients were enrolled in the study. Cardiac iron toxicity (ie, T2* < 20 ms) was seen in 28 (31%) patients; whereas in 62 (69%) patients, the cardiac iron level was undetectable (ie, T2* > 20 ms). Patients with T2* < 20 ms had significantly higher serum ferritin levels than those with T2* > 20 ms (P = 0.02). No significant correlation was found between the serum ferritin level and T2* (r = 0.08, P = 0.41). The left ventricular ejection fraction was statistically similar in the 2D and 3D examinations. Left atrial endsystolic and end-diastolic volumes were greater in the patients with iron cardiotoxicity than in those with no detectable cardiac iron deposition (P = 0.01 and P <0.001, respectively). Left atrial strain was also significantly lower in the patients with critical iron overload. The patients with T2* < 20 ms also had lower left atrial ejection fractions than those with T2* >20 ms, both in 2D and 3D examinations (both Ps <0.001).
Conclusions: Our study showed that changes in the left atrial structure and function precede impairment in the left ventricular systolic function in thalassemia patients with critical myocardial iron loading.

Keywords


1. Engle MA, Erlandson M, Smith CH. Late
cardiac complications of chronic, severe,
refractory anemia with hemochromatosis.
Circulation 1964;30:698705.
2. Olivieri NF. The beta-thalassemias. N Engl J
Med 1999;341:99109.
3. Borgna-Pignatti C, Rugolotto S, De Stefano
P, et al. Survival and complications in
patients with thalassemia major treated with
transfusion and desferrioxamine.
Haematologica 2004;89: 11871193.

4. Weatherall DJ, Clegg JB. Thalassemia a
global public health problem. Nat Med.
1996;2(8):847-9.
5. Westwood MA, Firmin DN, Gildo M, Renzo
G, Stathis G, Markissia K, et al. Intercentre
reproducibility of magnetic resonance T2*
measurements of myocardial in
thalassaemia. Int J Cardiovasc Imaging.
2005;21(5):531-8.
6. Modell B, Khan M, Darlison M. Survival in
beta-thalassemia major in UK: data from the
UK thalassemia register. Lancet.
2000;355:2051-2052
7. Hahalis G, Alexopoulos D, Kremastinos
DT, Zoumbos NC. Heart failure in β-
thalassemia syndromes: a decade of
progress. Am J Med, 118 (2005), pp. 957-
967
8. Aessopos A, Farmakis D, Berdoukas
V. Cardiac failure in β-thalassemia:
diagnosis, prevention and
management. Thalassemia
Reports 2011;1(1):59-65.
9. Wood JC. Impact of iron assessment by
MRI. Hematology Am Soc Hematol Educ
Program Book 2011 ;2011(1):443-450.
10. Anderson LJ, Holden S, Davis B, Prescott E,
Charrier CC, Bunce NH, Firmin DN, Wonke
B, Porter J, Walker JM, Pennell DJ:
Cardiovascular T2 star (T2*) magnetic
resonance for the early diagnosis of
myocardial iron overload. Eur Heart J 2001,
22:2171-2179.
11. Rodrigues A, Guimarães-Filho FV, Braga
JC, Rodrigues CS, Waib P, Fabron-Junior
A, Tan DM, França AC, Okoshi MP, Okoshi
K. Echocardiography in thalassemic patients
on blood transfusions and chelation without
heart failure. Arq Bras
Cardiol. 2013;100(1):75-81.
12. Arbab-Zadeh A, Dijk E, Prasad A, Fu Q,
Torres P, Zhang R, et al. Effect of aging and
physical activity on left ventricular
compliance. Circulation 2004;110:1799
805.
13. Sievers B, Kirchberg S, Addo M, Bakan A,
Brandts B, Trappe HJ. Assessment of left
atrial volumes in sinus rhythm and atrial
fibrillation using the biplane area-length
method and cardiovascular magnetic
resonance imaging with TrueFISP. J
Cardiovasc Magn Reson 2004;6:85563.
14. Kremastinos DT, Tsiapras DP, Tsetsos GA,
Rentoukas EI, Vretou HP, Toutouzas PK.
Left ventricular diastolic Doppler
characteristics in β-thalassemia major.
Circulation 1993;88:112735.
15. Westwood MA, Wonke B, Maceira AM,
Prescott E, Walker JM, Porter JB, et al. Left
ventricular diastolic function compared with
T2* cardiovascular magnetic resonance for
early detection of myocardial iron overload
in thalassemia major. J Magn Reson Imaging
2005;22:22933.
16. Aggeli A, Felekos I, Poulidakis E, Aggelis
A, Tousoulis D. Quantitative analysis of left
atrial function in asymptomatic patients with
b-thalassemia major using real-time threedimensional echocardiography. Cardiovasc
Ultrasound. 2011; 9: 38.