Kocaeli Med J. 2018; 7(1): 47-51 | DOI: 10.5505/ktd.2018.44265

Assessment of cardiac functions in pediatric patients with beta thalassemia major

Onur Çaglar Acar¹, Serdar Epçaçan², Ýbrahim Ece³, Abdurrahman Üner⁴, Ahmet Faik Öner^5
1Derince Education and Research Hospital, Department of Pediatric Cardiology, Kocaeli, Turkey
²Van Education and Research Hospital, Department of Pediatric Cardiology, Van, Turkey
³Türkiye Yüksek Ýhtisas Training and Research Hospital, Department of Pediatric Cardiology, Ankara, Turkey
⁴Bayrampasa Kolan Hospital, Department of Pediatric Cardiology, Istanbul, Turkey.
⁵Yuzuncu Yil University, Faculty of Medicine, Department of Pediatric Hematology, Van, Turkey

INTRODUCTION: Thalassemias are hereditary diseases caused by defects in the globulin chain synthesis with variable clinical severity. Cardiac dysfunction related to iron loading despite chelation treatment is a major cause of morbidity and mortality in these patients. The aim of this study is the evaluation of cardiac functions in beta thalassemia major diagnosed patients receiving oral chelation treatment.
METHODS: This study was performed on 49 cases between 2-16 years followed due to the diagnosis of beta thalassemia major in outpatient clinic between the dates of January-September 2014. 49 cases with similar age, gender, height and weight with the study group were selected as the control group. All cases underwent regular outpatient examination (physical examination, electrocardiography and echocardiography). Systolic and diastolic functions of the study and control groups were evaluated with echocardiography.
RESULTS: Average ferritin value of the study group was found to be 1275+904 ng/ml. In comparison of echocardiographic findings of the study group and control group, myocardial performance index was found to be significantly high in the study group (0.32 versus 0.43, p=0.017). In addition, left ventricular type 1 diastolic dysfunction was detected in the study group. No statistically significant difference was detected between two groups in terms of the left ventricular systolic, right ventricular systolic and diastolic functions.
DISCUSSION AND CONCLUSION: Our study indicates in patients diagnosed with beta thalassemia major that left ventricular diastolic dysfunction occurs at early age and the left ventricular systolic, right ventricular systolic and diastolic functions are not affected in early stage.

Keywords: Beta thalassemia major, myocardial performance index, diastolic dysfunction

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Beta Talesemi major tanýlý çocuk hastalarda kardiyak fonksiyonlarýn deðerlendirilmesi

Onur Çaglar Acar¹, Serdar Epçaçan², Ýbrahim Ece³, Abdurrahman Üner⁴, Ahmet Faik Öner^5
1Derince Eðitim ve Araþtýrma Hastanesi, Pediyiatrik Kardiyoloji Kliniði, Kocaeli
²Van Eðitim ve Araþtýrma Hastanesi, Pediyiatrik Kardiyoloji, Van
³Türkiye Yüksek Ýhtisas Eðitim ve Araþtýrma Hastanesi, Pediyatrik Kardiyoloji Kliniði, Ankara
⁴Bayrampaþa Kolan Hastanesi, Pediyatrik Kardiyoloji Kliniði, Ýstanbul
⁵Yüzüncü Yýl Üniversitesi Týp Fakültesi, Pediyatrik Hematoloji Bilim Dalý, Van

GÝRÝÞ ve AMAÇ: Talasemiler, globulin zincir sentezinde defektler sonucu oluþan kalýtsal hastalýklardýr. Bu hastalarda, þelasyon tedavisine raðmen demir birikimi sonucu oluþan kardiyak disfonksiyon önemli mortalite ve morbidite nedenidir. Kardiyak disfonksiyonun nedenleri kronik hemolitik anemi iliþkili ventrikül kaslýma disfonksiyonu ve kardiyak output artýþý ile birlikte ventriküler dilatasyon ve demir birikimidir. Bu çalýþmanýn amacý oral þelasyon tedavisi alan beta talasemi majör tanýlý hastalarda kardiyak fonksiyonlarýn Doppler ekokardiyografi ile incelenmesidir.
YÖNTEM ve GEREÇLER: Bu çalýþmada, Ocak-Eylül 2014 tarihleri arasýnda, beta talasemi major tanýlý, 2-16 yaþ arasýnda 49 hasta ve kontrol grubu olarak da ayný sayýda 49 saðlýklý hasta incelendi. Tüm hastalara ayrýntýlý fizik muayane sonrasýnda elektrokardiyografik ve ekokardiyografik inceleme uygulandý.
BULGULAR: Hasta grubunun ortalama ferritin deðeri 1275+904 ng/ml idi. Gruplarýn ekokardiyografik bulgularý karþýlaþtýrýldýðýnda, hasta grubunda miyokardiyal performans indeksi istatistiksel olarak yüksek bulundu (p=0.017). Ek olarak, hasta grubunda sol ventrikülde tip 1 diyastolik disfonksiyon saptanýrken, sol ventrikül sistolik, sað ventrikül sistolik ve diyastolik disfonksiyon görülmedi.
TARTIÞMA ve SONUÇ: Talasemi majör tanýlý hastalarda baþta kardiyovaskuler sistem olmak üzere birçok sistemde demir birikimi toksik etki göstermektedir. Demir þelasyon tedavisi ile hastalýðýn prognozu düzelse de yüksek moratalite ve morbidite halen önemli bir problem olarak kalmaktadýr. Çalýþmamýz beta talasemi majorlu hastalarda erken dönemde subklinik sol ventrikül diyastolik disfonksiyonu geliþtiðini, buna karþýn sað ventrikül fonksiyonlarýnýn ve sol ventrikül sistolik fonksiyonlarýnýn bozulmadýðýný göstermektedir.

Anahtar Kelimeler: Beta talasemi major, miyokardiyal performans indeks, diyastolik disfonksiyon.

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