Talasemi Majör Hastalarında MRG ile Kardiyak Demir Yükü Ölçümü, Demir Yükünün Kardiyak Fonksiyonlara Etkisinin Değerlendirilmesi ve Doku Doppleri ile Karşılaştırılması

Yıl 2024, , 81 - 96, 30.04.2024

Tevfik Güzelbey , Bengi Gürses , Aysegül Sarsılmaz , Ebru Karasu , Olcay Özveren , Yusuf Sinan Aydın

https://doi.org/10.38079/igusabder.1362022

Cited By: 1

Öz

Amaç: Talasemi majör (TM) hastalarında, düzenli kan transfüzyonları nedeniyle demir birikimi görülmektedir. TM hastalarında morbidite ve mortalitenin en sık nedeni artan demir birikimine bağlı gelişen kardiyak komplikasyonlardır. Kardiyak demir birikimi manyetik rezonans görüntüleme (MRG) ile doğru bir şekilde ölçülebilmektedir. Ancak 3 Tesla (3T) MRG ile gerçekleştirilmiş yeterince çalışma bulunmamaktadır. Bu çalışmanın amacı, kardiyak demir birikimini 3T MRG cihazında T2* relaksasyon tekniği ile ölçmek ve kardiyak T2* değerleri ile karaciğer T2* değerlerinin, serum ferritin seviyesinin, ekokardiyografi ve kardiyak MRG (KMRG)’de sistolik ve diyastolik fonksiyonları değerlendirmek için ölçülen parametrelerin ilişkisini araştırmaktır.
Yöntem: 21 TM hastasına (13 kadın, 8 erkek; ortalama yaş 29,3±7,6) ve 11 sağlıklı gönüllüye (5 kadın, 6 erkek; ortalama yaş 32,4±5,9) kardiyoloji departmanı tarafından transtorasik ekokardiyografi (TTE) yapıldı. TTE’den sonra bir hafta içerisinde kardiyak ve karaciğer demir yükünü değerlendirmek için 3T MRG cihazında 16 ekolu T2* gradiyent Turbo Field Echo (TFE) sekansı kullanıldı. Kardiyak fonksiyonları değerlendirmek amacı ile 2D-Balanced (steady-state free precession)-TFE sekansı ile kısa aks sine görüntüler alındı.
Bulgular: 21 TM hastasının 6’sında kardiyak T2* değeri 12 msn altında ölçüldü. Kardiyak demir birikimi olan ve olmayan TM hastalarında karaciğer T2* değeri, serum ferritin düzeyi, TTE ve MRG’de sistolik ve diyastolik fonksiyonların değerlendirilmesinde kullanılan parametreler arasında anlamlı fark saptanmadı (p>0,05). Ayrıca kardiyak T2* değeri ile ölçülen diğer tüm parametreler arasında anlamlı korelasyon ilişkisi bulunmadı (p>0,05). Serum ferritin düzeyi ile karaciğer T2* değeri arasında orta düzeyde negatif korelasyon saptandı (r = -0,663). TTE’de sistolik veya diyastolik disfonksiyon lehine bulgu bulunmadı.
Sonuç: Kardiyak demir yükünü öngörmede TTE ve KMRG’de ölçülen parametrelerin, serum ferritin düzeyinin ve karaciğer T2* değerinin yeri bulunmamaktadır. TM hastalarının kardiyak demir birikimi ve birikime yönelik şelasyon tedavisi takibinde kardiyak T2* değeri kullanılmalıdır.

Anahtar Kelimeler

Talasemi majör, manyetik rezonans görüntüleme, demir aşırı yükü

Measurement of Cardiac Iron Load with MRI in Thalassemia Major Patients, Evaluation of the Effect of Iron Load on Cardiac Functions, and Comparison with Tissue Doppler

Öz

Aim: Thalassemia major (TM) patients typically suffer from iron overload due to regular blood transfusions, with cardiac complications representing the primary causes of morbidity and mortality in TM. Magnetic resonance imaging (MRI) has gained prominence in the quantitative assessment of myocardial iron overload. However, the quantification of cardiac iron using 3 Tesla MRI has not been thoroughly explored in existing literature. This study aims to quantify cardiac iron load using 3T MRI, employing the T2* relaxation technique, and to investigate the association between cardiac T2* values and liver T2* values, serum ferritin levels, as well as systolic and diastolic functions assessed by echocardiography (ECHO) and MRI.
Method: Transthoracic echocardiography (TTE) was performed on 21 TM patients (13 females, 8 males; mean age 29.3±7.6) and 11 healthy volunteers (5 females, 6 males; mean age 32.4±5.9) by the cardiology department. Within one week following the TTE, a 16-echo T2* gradient Turbo Field Echo (TFE) sequence was used in a 3T MRI device to assess cardiac and liver iron load. Short-axis cine images were obtained using a 2D-Balanced (steady-state free precession)-TFE sequence for the evaluation of cardiac functions.
Results: Of the 21 TM patients, 6 showed cardiac T2* values below 12 ms. No significant difference was observed between TM patients with and without cardiac iron accumulation in terms of liver T2* values, serum ferritin levels, and parameters used to evaluate systolic and diastolic functions in TTE and MRI (p>0.05). Additionally, no significant correlation was found between cardiac T2* values and all other measured parameters (p>0.05). A moderate negative correlation was detected between serum ferritin levels and liver T2* values (r = -0.663). No signs favoring systolic or diastolic dysfunction were found in TTE.
Conclusion: The parameters measured in TTE and CMRI, serum ferritin levels, and liver T2* values do not have a role in predicting cardiac iron load. The cardiac T2* value should be used in the monitoring of cardiac iron accumulation and chelation therapy in TM patients.

Anahtar Kelimeler

Thalassemia major, magnetic resonance imaging, iron overload

Kaynakça

• 1. Galanello R, Origa R. Beta-thalassemia. Orphanet J Rare Dis. 2010;5:11. doi: 10.1186/1750-1172-5-11.
• 2. Weatherall DJ. The inherited diseases of hemoglobin are an emerging global health burden. Blood. 2010;115(22):4331-6. doi: 10.1182/blood-2010-01-251348.
• 3. Anderson LJ, Holden S, Davis B, et al. Cardiovascular T2-star (T2*) magnetic resonance for the early diagnosis of myocardial iron overload. Eur Heart J. 2001;22(23):2171-9. doi: 10.1053/euhj.2001.2822.
• 4. Westwood MA, Wonke B, Maceira AM, 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(2):229-33. doi: 10.1002/jmri.20379.
• 5. Messroghli DR, Bainbridge GJ, Alfakih K, et al. Assessment of regional left ventricular function: accuracy and reproducibility of positioning standard short-axis sections in cardiac MR imaging. Radiology. 2005;235(1):229-36. doi: 10.1148/radiol.2351040249.
• 6. Guo H, Au WY, Cheung JS, et al. Myocardial T2 quantitation in patients with iron overload at 3 Tesla. J Magn Reson Imaging. 2009;30(2):394-400. doi: 10.1002/jmri.21851.
• 7. Okayama S, Nakano T, Uemura S, et al. Evaluation of left ventricular diastolic function by fractional area change using cine cardiovascular magnetic resonance: a feasibility study. J Cardiovasc Magn Reson. 2013;15(1):87. doi: 10.1186/1532-429X-15-87.
• 8. Sahn DJ, DeMaria A, Kisslo J, Weyman A. Recommendations regarding quantitation in M-mode echocardiography: results of a survey of echocardiographic measurements. Circulation. 1978;58(6):1072-83. doi: 10.1161/01.cir.58.6.1072.
• 9. Nagueh SF, Appleton CP, Gillebert TC, et al. Recommendations for the evaluation of left ventricular diastolic function by echocardiography. J Am Soc Echocardiogr. 2009;22(2):107-33. doi: 10.1016/j.echo.2008.11.023.
• 10. Gandon Y, Olivie D, Guyader D, et al. Non-invasive assessment of hepatic iron stores by MRI. Lancet. 2004;363(9406):357-62. doi: 10.1016/S0140-6736(04)15436-6.
• 11. Auger D, Pennell DJ. Cardiac complications in thalassemia major. Ann N Y Acad Sci. 2016;1368(1):56-64. doi: 10.1111/nyas.13026.
• 12. Wood JC. Use of magnetic resonance imaging to monitor iron overload. Hematol Oncol Clin North Am. 2014;28(4):747-64, vii. doi: 10.1016/j.hoc.2014.04.002.
• 13. Aessopos A, Giakoumis A, Fragodimitri C, et al. Correlation of echocardiography parameters with cardiac magnetic resonance imaging in transfusion-dependent thalassaemia major. Eur J Haematol. 2007;78(1):58-65. doi: 10.1111/j.1600-0609.2006.00770.x.
• 14. Alexopoulou E, Stripeli F, Baras P, et al. R2 relaxometry with MRI for the quantification of tissue iron overload in beta-thalassemic patients. J Magn Reson Imaging. 2006;23(2):163-70. doi: 10.1002/jmri.20489.
• 15. Pennell DJ. T2* magnetic resonance and myocardial iron in thalassemia. Ann N Y Acad Sci. 2005;1054:373-8. doi: 10.1196/annals.1345.045.
• 16. Khadivi Heris H, Nejati B, Rezazadeh K, et al. Evaluation of iron overload by cardiac and liver T2* in beta-thalassemia: Correlation with serum ferritin, heart function and liver enzymes. J Cardiovasc Thorac Res. 2021;13(1):54-60. doi: 10.34172/jcvtr.2021.18.
• 17. Shamsian BS, Esfahani SA, Milani H, et al. Magnetic resonance imaging in the evaluation of iron overload: a comparison of MRI, echocardiography and serum ferritin level in patients with beta-thalassemia major. Clin Imaging. 2012;36(5):483-8. doi: 10.1016/j.clinimag.2011.11.029.
• 18. Pennell D. MRI and iron-overload cardiomyopathy in thalassaemia. Circulation. 2006;113(11):f43-4.
• 19. Kirk P, Roughton M, Porter JB, et al. Cardiac T2* magnetic resonance for prediction of cardiac complications in thalassemia major. Circulation. 2009;120(20):1961-8. doi: 10.1161/CIRCULATIONAHA.109.874487.
• 20. Westwood MA, Anderson LJ, Maceira AM, et al. Normalized left ventricular volumes and function in thalassemia major patients with normal myocardial iron. J Magn Reson Imaging. 2007;25(6):1147-51. doi: 10.1002/jmri.20915.
• 21. Bosi G, Crepaz R, Gamberini MR, et al. Left ventricular remodelling, and systolic and diastolic function in young adults with beta thalassaemia major: a Doppler echocardiographic assessment and correlation with haematological data. Heart. 2003;89(7):762-6. doi: 10.1136/heart.89.7.762.
• 22. Seliem MA, Al-Saad HI, Bou-Holaigah IH, Khan MN, Palileo MR. Left ventricular diastolic dysfunction in congenital chronic anaemias during childhood as determined by comprehensive echocardiographic imaging including acoustic quantification. Eur J Echocardiogr. 2002;3(2):103-10. doi: 10.1053/euje.2001.0122.
• 23. Hou JW, Wu MH, Lin KH, Lue HC. Prognostic significance of left ventricular diastolic indexes in beta-thalassemia major. Arch Pediatr Adolesc Med. 1994;148(8):862-6. doi: 10.1001/archpedi.1994.02170080092018.
• 24. Gharzuddine WS, Kazma HK, Nuwayhid IA, et al. Doppler characterization of left ventricular diastolic function in beta-thalassaemia major. Evidence for an early stage of impaired relaxation. Eur J Echocardiogr. 2002;3(1):47-51. doi: 10.1053/euje.2001.0114.