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Left atrial deformation indices in β-thalassemia major patients

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Abstract

The presence of atrial cardiomyopathy in β-thalassemia major (β-TM) patients complicates their clinical condition. The diagnosis is challenging even with cardiac magnetic resonance (CMR) imaging. Novel echocardiographic techniques are applied to increase the diagnostic yield. Fifty-six β-TM patients and thirty age and sex-matched controls were included in the present cross-sectional study. Heart rate, PR duration, and P axis were measured by electrocardiography, left ventricular ejection fraction (LVEF) and end-diastolic diameter (LVEDD), ratio between early mitral inflow velocity and mitral annular early diastolic velocity (E/e′), left atrial volume index (LAVI), left atrial strain at reservoir (LASr), conduit (LAScd) and contraction (LASct) phases respectively, left ventricular global longitudinal strain (GLS) by echocardiography, and T2* calculation in patient group by CMR. PR duration, LVEF, LAVI, E/e′, GLS, and left atrial deformation parameters differed between patients and controls (p <0.05). In patient group, left atrial strain was correlated with PR duration, LAVI, E/e′, GLS, and T2* (p <0.05). T2* was correlated only with left atrial deformation indices (p <0.05). Patients with a history of atrial fibrillation were older, had lower heart rate, prolonged PR, increased E/e′ and LAVI, and impaired left atrial strain (p <0.05). LASct differed relative to the presence of atrial fibrillation and myocardial iron overload. Atrial strain could be of clinical use in the early detection of atrial cardiomyopathy. An impaired LASct could identify β-TM patients with undetected episodes of atrial fibrillation. Finally, left atrial strain may be helpful in myocardial iron load estimation.

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Data availability

The datasets generated during and/or analysed during the current study are not publicly available, but are available from the corresponding author on reasonable request.

Abbreviations

CMR:

Cardiac magnetic resonance

E/e′:

Ratio between early mitral inflow velocity and mitral annular early diastolic velocity

GLS :

Global longitudinal strain of left ventricle

LAScd :

Left atrial strain at conduit phase

LASct :

Left atrial strain at contraction phase

LASr :

Left atrial strain at reservoir phase

LAVI :

Left atrial volume index

LVEDD :

Left ventricular end-diastolic diameter

LVEF :

Left ventricular ejection fraction

References

  1. Higgs DR, Engel JD, Stamatoyannopoulos G (2012) Thalassaemia. Lancet. 379(9813):373–383. https://doi.org/10.1016/S0140-6736(11)60283-3

    Article  CAS  PubMed  Google Scholar 

  2. Williams TN, Weatherall DJ (2012) World distribution, population genetics, and health burden of the hemoglobinopathies. Cold Spring Harb Perspect Med. 2(9):a011692. https://doi.org/10.1101/cshperspect.a011692

    Article  PubMed  PubMed Central  Google Scholar 

  3. Pennell DJ, Udelson JE, Arai AE, Bozkurt B, Cohen AR, Galanello R et al (2013) American Heart Association Committee on Heart Failure and Transplantation of the Council on Clinical Cardiology and Council on Cardiovascular Radiology and Imaging. Cardiovascular function and treatment in β-thalassemia major: a consensus statement from the American Heart Association. Circulation 128(3):281-308. https://doi.org/10.1161/CIR.0b013e31829b2be6. Erratum in: Circulation. 2013 Sep 24;128(13):e203. https://doi.org/10.1161/CIR.0b013e3182a72d1c

  4. Krittayaphong R, Viprakasit V, Saiviroonporn P, Siritanaratkul N, Siripornpitak S, Meekaewkunchorn A et al (2017) Prevalence and predictors of cardiac and liver iron overload in patients with thalassemia: a multicenter study based on real-world data. Blood Cells Mol Dis. 66:24–30. https://doi.org/10.1016/j.bcmd.2017.08.002

    Article  CAS  PubMed  Google Scholar 

  5. Wijarnpreecha K, Kumfu S, Chattipakorn SC, Chattipakorn N (2015) Cardiomyopathy associated with iron overload: how does iron enter myocytes and what are the implications for pharmacological therapy? Hemoglobin. 39(1):9–17. https://doi.org/10.3109/03630269.2014.987869

    Article  CAS  PubMed  Google Scholar 

  6. Ballas SK, Zeidan AM, Duong VH, DeVeaux M, Heeney MM (2018) The effect of iron chelation therapy on overall survival in sickle cell disease and β-thalassemia: a systematic review. Am J Hematol. 93(7):943–952. https://doi.org/10.1002/ajh.25103

    Article  PubMed  Google Scholar 

  7. Kirk P, Roughton M, Porter JB, Walker JM, Tanner MA, Patel J et al (2009) Cardiac T2* magnetic resonance for prediction of cardiac complications in thalassemia major. Circulation. 120(20):1961–1968. https://doi.org/10.1161/CIRCULATIONAHA.109.874487

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Triadyaksa P, Oudkerk M, Sijens PE (2020) Cardiac T2 * mapping: Techniques and clinical applications. J Magn Reson Imaging. 52(5):1340–1351. https://doi.org/10.1002/jmri.27023

    Article  PubMed  Google Scholar 

  9. Kostopoulou AG, Tsiapras DP, Chaidaroglou AS, De Giannis DE, Farmakis D, Kremastinos DT (2014) The pathophysiological relationship and clinical significance of left atrial function and left ventricular diastolic dysfunction in β-thalassemia major. Am J Hematol. 89(1):13–18. https://doi.org/10.1002/ajh.23581

    Article  CAS  PubMed  Google Scholar 

  10. Li W, Coates T, Wood JC (2008) Atrial dysfunction as a marker of iron cardiotoxicity in thalassemia major. Haematologica. 93(2):311–312. https://doi.org/10.3324/haematol.11958

    Article  CAS  PubMed  Google Scholar 

  11. Badano LP, Kolias TJ, Muraru D, Abraham TP, Aurigemma G, Edvardsen T et al (2018) Standardization of left atrial, right ventricular, and right atrial deformation imaging using two-dimensional speckle tracking echocardiography: a consensus document of the EACVI/ASE/Industry Task Force to standardize deformation imaging. Eur Heart J Cardiovasc Imaging 19(6):591–600. https://doi.org/10.1093/ehjci/jey042. Erratum in: Eur Heart J Cardiovasc Imaging. 2018;19(7):830–833. https://doi.org/10.1093/ehjci/jey071

  12. Voigt JU, Pedrizzetti G, Lysyansky P, Marwick TH, Houle H, Baumann R et al (2015) Definitions for a common standard for 2D speckle tracking echocardiography: consensus document of the EACVI/ASE/Industry Task Force to standardize deformation imaging. J Am Soc Echocardiogr. 28(2):183–193. https://doi.org/10.1016/j.echo.2014.11.003

    Article  PubMed  Google Scholar 

  13. Cho GY, Hwang IC (2020) Left atrial strain measurement: a new normal for diastolic assessment? JACC Cardiovasc Imaging. 13(11):2327–2329. https://doi.org/10.1016/j.jcmg.2020.05.014

    Article  PubMed  Google Scholar 

  14. Potter EL, Ramkumar S, Kawakami H, Yang H, Wright L, Negishi T, Marwick TH (2020) Association of asymptomatic diastolic dysfunction assessed by left atrial strain with incident heart failure. JACC Cardiovasc Imaging. 13(11):2316–2326. https://doi.org/10.1016/j.jcmg.2020.04.028

    Article  PubMed  Google Scholar 

  15. Goette A, Kalman JM, Aguinaga L, Akar J, Cabrera JA, Chen SA et al (2016) EHRA/HRS/APHRS/SOLAECE expert consensus on atrial cardiomyopathies: definition, characterization, and clinical implication. Europace. 18(10):1455–1490. https://doi.org/10.1093/europace/euw161

    Article  PubMed  PubMed Central  Google Scholar 

  16. Ozdogan O, Alp A, Turker M, Atabey B (2013) Determination of early cardiac deterioration in beta-thalassaemia major by echocardiography. Acta Cardiol. 68(3):299–305. https://doi.org/10.1080/ac.68.3.2983425

    Article  PubMed  Google Scholar 

  17. Rodrigues A, Guimarães-Filho FV, Braga JC, Rodrigues CS, Waib P, Fabron-Junior A et al (2013) Echocardiography in thalassemic patients on blood transfusions and chelation without heart failure. Arq Bras Cardiol Jean;100(1):75-81. English, Portuguese. https://doi.org/10.1590/s0066-782x2013005000001

  18. Nomani H, Bayat G, Sahebkar A, Fazelifar AF, Vakilian F, Jomezade V et al (2019) Atrial fibrillation in β-thalassemia patients with a focus on the role of iron-overload and oxidative stress: a review. J Cell Physiol. 234(8):12249–12266. https://doi.org/10.1002/jcp.27968

    Article  CAS  PubMed  Google Scholar 

  19. Voskaridou E, Kattamis A, Fragodimitri C, Kourakli A, Chalkia P, Diamantidis M et al (2019) National registry of hemoglobinopathies in Greece: updated demographics, current trends in affected births, and causes of mortality. Ann Hematol. 98(1):55–66. https://doi.org/10.1007/s00277-018-3493-4

    Article  CAS  PubMed  Google Scholar 

  20. Gan GCH, Bhat A, Chen HHL, Gu KH, Fernandez F, Kadappu KK et al (2021) Left atrial reservoir strain by speckle tracking echocardiography: association with exercise capacity in chronic kidney disease. J Am Heart Assoc. 10(1):e017840. https://doi.org/10.1161/JAHA.120.017840

    Article  PubMed  Google Scholar 

  21. Meloni A, Maggio A, Positano V, Leto F, Angelini A, Putti MC et al (2020) CMR for myocardial iron overload quantification: calibration curve from the MIOT Network. Eur Radiol. 30(6):3217–3225. https://doi.org/10.1007/s00330-020-06668-1

    Article  PubMed  Google Scholar 

  22. Patsourakos D, Gatzoulis KA, Aggeli C, Delicou S, Dimitroglou Y, Xydaki K et al (2020) Twelve-lead and signal-averaged electrocardiographic parameters among beta-thalassemia major patients. J Arrhythm. 36(5):920–928. https://doi.org/10.1002/joa3.12412

    Article  PubMed  PubMed Central  Google Scholar 

  23. Shabanian R, Heidari-Bateni G, Kocharian A, Mashayekhi M, Hosseinzadeh S, Kiani A et al (2010) Augmentation of left atrial contractile function: a herald of iron overload in patients with beta thalassemia major. Pediatr Cardiol. 31(5):680–688. https://doi.org/10.1007/s00246-010-9693-x

    Article  PubMed  Google Scholar 

  24. Karamanou AG, Hamodraka ES, Vrakas SC, Paraskevaidis I, Lekakis I, Kremastinos DT (2014) Assessment of left ventricular and atrial diastolic function using two-dimensional (2D) strain imaging in patients with β-thalassemia major. Eur J Haematol. 92(1):59–65. https://doi.org/10.1111/ejh.12209

    Article  CAS  PubMed  Google Scholar 

  25. Cheung YF, So EK, Hwang GY, Chan GC, Ha SY (2019) Left and right atrial function and remodeling in beta-thalassaemia major. Pediatr Cardiol. 40(5):1001–1008. https://doi.org/10.1007/s00246-019-02105-3

    Article  PubMed  Google Scholar 

  26. Casale M, Meloni A, Filosa A, Cuccia L, Caruso V, Palazzi G et al (2015) Multiparametric cardiac magnetic resonance survey in children with thalassemia major: a multicenter study. Circ Cardiovasc Imaging. 8(8):e003230. https://doi.org/10.1161/CIRCIMAGING.115.003230

    Article  PubMed  Google Scholar 

  27. Barbero U, Fornari F, Guarguagli S, Gaglioti CM, Longo F, Doronzo B et al (2018) Atrial fibrillation in β-thalassemia major patients: diagnosis, management and therapeutic options. Hemoglobin. 42(3):189–193. https://doi.org/10.1080/03630269.2018.1488724

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. First Department of Cardiology, General Hospital of Athens Ippokrateio, National and Kapodistrian University of Athens, 114 Vasilissis Sofias Avenue, 11527, Attica, Athens, Greece

    Dimitrios Patsourakos, Constantina Aggeli, Konstantinos A. Gatzoulis, Yannis Dimitroglou & Konstantinos Tsioufis

  2. Thalassemia and Sickle Cell Unit, General Hospital of Athens Ippokrateio, 114 Vasilissis Sofias Avenue, 11527, Athens, Greece

    Sophia Delicou & Katerina Xydaki

  3. Thalassemia Unit, Agia Sophia Children’s Hospital, Thivon and Papadiamantopoulou Str., 11527, Athens, Greece

    Christina Fragodimitri

  4. State Department of Cardiology, General Hospital of Athens Ippokrateio, 114 Vasilissis Sofias Avenue, 11527, Athens, Greece

    Aristeidis Androulakis

Authors
  1. Dimitrios Patsourakos
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  2. Constantina Aggeli
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  3. Konstantinos A. Gatzoulis
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Contributions

D. P. and C. A. are responsible for the recruitment and medical examination. C.A. conceived the design of the study and D. P. wrote the manuscript. S. D., K. X., and C. F. referred the patients and provided information regarding patient hematological history. Y. D. has contributed in statistical analysis and off-line strain processing. K. A. G., A. A., and K. T. thoroughly reviewed and commented on the manuscript.

Corresponding author

Correspondence to Dimitrios Patsourakos.

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The human research and bioethics committee of the affiliated health institution has granted permission for the current study.

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Informed consent was obtained from all participants in the present study, which conforms to the ethical guidelines of the 1975 Declaration of Helsinki and complies with the principles of General Data Protection Regulation (GDPR).

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Informed consent was obtained from all participants in the present study for publication with respect to anonymity.

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The authors declare no competing interests.

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Dimitrios Patsourakos, Constantina Aggeli, Konstantinos A. Gatzoulis, Sophia Delicou, Yannis Dimitroglou, Katerina Xydaki, Christina Fragodimitri, Aristeidis Androulakis, and Konstantinos Tsioufis take responsibility for all aspects of the reliability and freedom from bias of the data presented and their discussed interpretation.

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Patsourakos, D., Aggeli, C., Gatzoulis, K.A. et al. Left atrial deformation indices in β-thalassemia major patients. Ann Hematol 101, 1473–1483 (2022). https://doi.org/10.1007/s00277-022-04842-7

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