TALASEMİ MAJORLÜ ÇOCUK VE GENÇ YETİŞKİNLERDE DEMİR YÜKÜ İLE YAŞ VE KARDİYAK FONKSİYONLARIN İLİŞKİSİNİN DEĞERLENDİRİLMESİ: BİR DARBELİ DOPPLER VE DOKU DOPPLER GÖRÜNTÜLEME ÇALIŞMASI

Abstract

Amaç: Darbeli dalga Doppler ve doku Doppler görüntüleme yöntemleri kullanılarak talasemi majorlu (TM) çocuk ve genç yetişkinlerde sağlıklı kişilere göre kardiyak yapısal ve fonksiyonel farklılıklarının belirlenmesi; demir yükünün bu farklılıklar ile olan ilişkisinin ortaya konması amaçlanmıştır. Gereç ve Yöntem: Çalışmamızı yaşları 4-22 arasında olan çocuk ve genç yetişkin TM lü hastaların (n=44) ve yaş-cinsiyet uyumlu kontrol grubunun (n=40) hastanemiz veri sistemindeki 01.10.2023 ile 01.10.2024 tarihleri arasındaki verilerinin incelenmesiyle oluşturduk. Hastaların boy, kilo, vücut kitle indexi (VKI), sistolik-diyastolik kan basıncı ölçümleri ile tam kan sayımı, ferritin, kardiyak T2*MRI değerleri ve ekokardiyografi kayıtları kaydedildi. İki grup arasında karşılaştırmaların yanısıra; ve TM hastalarında ferritin-kardiyak T2*MRI sonuçları ile ekokardiyografik parametreler-yaş arasında korelasyon analizi yapıldı. Bulgular: Çalışmamızda TM hastalarında büyüme gelişme geriliği (boy SDS, kilo SDS ve VKI SDS düşük), sol boşluklarda dilatasyon (LVIDd yüksek), sol ventrikül kas kitlesinde artış (LVMI yüksek), kardiyak aşikar diyastolik (restriktif özellikte olan: LV tepe erken diyastolik akımı (E)/tepe geç diyastolik akımı (A) ve E/erken diyastolik miyokardiyal tepe akımı (E') yüksek) ve subklinik sistolik (LV tepe sistolik akımı (Sm) düşük ve LV Tei indexi (TX) yüksek) fonksiyon bozukluğu olduğunu tespit ettik. Bununla birlikte demir yükü (ferritin ve kardiyak T2*MRI) ile LVMI, kardiyak diyastolik ve sistolik fonksiyon göstergeleri arasında ilişki mevcuttu. Yaş arttıkça ferritin değeri değişmezken kardiyak T2*MRI değerinin azaldığı; diyastolik ve sistolik parametrelerde kötüleşmelerin olduğu tespit edildi. Sonuç: TM lü hastaların periyodik olarak kardiyak T2*MRI ile doppler ekokardiyografi incelemelerinin yapılmasıyla subklinik myokardiyal disfonksiyon erken dönemde tespit edilebilir; bu da bize gerekli müdahelelerin yapılması açısından fırsat penceresi sunabilir.

Keywords

• talasemi
• kardiyak fonksiyonlar
• aşırı demir yükü
• kardiyak T2 MR,
• Doppler ekokardiyografi

EVALUATION OF THE RELATIONSHIP BETWEEN IRON LOAD, AGE, AND CARDIAC FUNCTION IN CHILDREN AND YOUNG ADULTS WITH THALASSEMIA MAJOR: A PULSED-WAVE DOPPLER AND TISSUE DOPPLER IMAGING STUDY

Abstract

Objective: To determine the structural and functional cardiac differences in children and young adults with thalassemia major (TM) compared to healthy subjects using pulsed-wave Doppler and tissue Doppler imaging methods and determine the relationship between iron overload and these differences. Materials and Methods: We analyzed the data of pediatric and young adult TM patients (n = 44) aged 4–22 years and an age- and gender-matched control group (n = 40) in our hospital data system between Oct.01.2023 and Oct.01.2024. Height, weight, body mass index (BMI), systolic–diastolic blood pressure measurements, complete blood count, ferritin, cardiac T2* magnetic resonance imaging (MRI) values, and echocardiography results were recorded. In addition to comparisons between the two groups, correlation analysis was performed between ferritin–cardiac T2* MRI results and echocardiographic parameters and age in TM patients. Results: Our study showed growth retardation (low height standard deviation score (SDS), low weight SDS and low BMI SDS), dilatation of the left cavities (high left ventricular internal diameter end diastole (LVIDd)), increased left ventricular muscle mass (high left ventricular mass index (LVMI)), cardiac distinctive diastolic (restrictive pattern: left ventricular (LV) peak early diastolic flow (E)/peak late diastolic flow (A) and E/early diastolic myocardial peak flow (E') high), and subclinical systolic (LV peak systolic flow low and LV Tei index high) dysfunction. In addition, iron load (ferritin and cardiac T2* MRI) was correlated with LVMI, and cardiac diastolic and systolic function indicators. As age increased, ferritin value did not change, but cardiac T2* MRI value decreased and diastolic–systolic parameters worsened. Conclusion: Periodic cardiac T2* MRI and Doppler echocardiography examinations of patients with TM may detect subclinical myocardial dysfunction at an early stage, thus providing a window of opportunity for intervention.

Keywords

• thalassemia
• cardiac functions
• iron overload
• cardiac T2 MRI
• Doppler echocardiography

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