Tesis

Peran speckle tracking echocardiography dalam deteksi disfungsi ventrikel asimtomatik pada anak talasemia mayor = The role of speckle tracking echocardiography in detecting asymptomatic ventricular dysfunction in children with thalassemia major.

Latar Belakang: Gagal jantung merupakan penyebab utama kematian pasien talasemia mayor (TM). Angka mortalitas global pada pasien TM mencapai 70%, tetapi sering tidak bergejala pada tahap awal dan berobat terlambat ketika sudah menderita gagal jantung. Ekokardiografi konvensional sering luput mendeteksi tanda awal disfungsi ventrikel pasien TM. Kami meneliti peran speckle tracking echocardiography (STE) dalam mendeteksi disfungsi ventrikel asimtomatik pada pasien TM. Metode: Uji diagnostik speckle tracking echocardiography dilakukan pada 54 pasien anak talasemia mayor dalam mendeteksi disfungsi ventrikel. Analisis multivariat dilakukan untuk melihat peran faktor risiko usia, lama transfusi, kadar feritin dan saturasi transferin dalam kejadian disfungsi ventrikel. Hasil: Didapatkan 28 subyek (52%) disfungsi diastolik dan 22 subyek (41%) disfungsi sistolik. Kadar feritin terbukti berperan dalam kejadian disfungsi diastolik dan disfungsi sistolik ventrikel kiri, dengan nilai rerata berturut-turut 6662 ng/ml (p=0,032) dan 7176 ng/ml (p=0,005). Nilai titik potong untuk kadar feritin yang berhubungan dengan disfungsi ventrikel adalah 5624 ng/ml. Dengan nilai AUC pada disfungsi diastolik 0,675% (IK95% 0,531–0,820) dan nilai AUC pada disfungsi sistolik 0,702% (IK95% 0,553–0,852). Penilaian STE terhadap kejadian disfungsi diastolik memiliki sensitivitas moderat 61% dan spesifisitas yang rendah 46%. Sedangkan pada kejadian disfungsi sistolik memiliki sensitivitas tinggi 91% dan spesifisitas moderat 66%. Kesimpulan: Dari 54 pasien TM yang tampak sehat, 28 pasien mengalami disfungsi diastolik dan 22 pasien mengalami disfungsi sistolik. Faktor risiko kejadian disfungsi ventrikel pada pasien TM adalah kadar feritin ≥ 5624 ng/ml. Speckle tracking echocardiography dapat mendeteksi disfungsi diastolik dan disfungsi sistolik ventrikel kiri pada pasien dengan LVEF yang normal.
Kata kunci: Talasemia mayor, Disfungsi ventrikel, Feritin, Speckle tracking echocardiography


Background: End-stage heart failure due to iron overload cardiomyopathy are the major cause of mortality in thalassemia major (TM) patients, but many patients remain asymptomatic until the late stage. Detection of ventricular dysfunction using conventional echocardiography often misses the early signs of ventricular dysfunction. We investigated the role of speckle tracking echocardiography (STE) in detecting asymptomatic ventricular dysfunction in TM patients. Methods: A diagnostic test of speckle tracking echocardiography conducted in detecting ventricular dysfunction in children with TM. Multivariate analysis assessed the relationship between age, duration of transfusion, ferritin levels, transferrin saturation and ventricular dysfunction. were performed to evaluate the role of each risk factor. Results: The number of research subjects were 54 subjects, the median age being 14 (8–18) years. From the study, 28 subjects (52%) had diastolic dysfunction and 22 subjects (41%) had systolic dysfunction. Only ferritin levels were proven to play a role in the incidence of left ventricular diastolic dysfunction and systolic dysfunction, with the cut off point for ferritin levels associated with ventricular dysfunction was 5624.50 ng/ml. With a fairly good AUC value, namely for diastolic dysfunction was 0.675% (CI 95% 0.531–0.820) and the AUC value for systolic dysfunction was 0.702% (95% CI 0.553–0.852). Diastolic dysfunction assessed by STE has a moderate sensitivity of 61% and a low specificity of 46%. Meanwhile, the incidence of systolic dysfunction has a high sensitivity of 91% and a moderate specificity of 66%. Conclusions: From 54 apparently healthy TM patients, 28 patients had developed diastolic dysfunction and 22 had systolic dysfunction. Ferritin has been shown to be a significant factor in the incidence of ventricular dysfunction with cut-off point value of 5265.5 ng/ml. Speckle tracking echocardiography can detect left ventricular diastolic and systolic dysfunction in patients with normal LVEF.
Keywords: Thalassemia major, Ventricular dysfunction, Speckle tracking echocardiography