Evaluation of the North Star Ambulatory Assessment scale and cardiac abnormalities in ambulant boys with Duchenne muscular dystrophy |
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Authors: | Ergul Yakup Ekici Baris Nisli Kemal Tatli Burak Binboga Fatih Acar Gonul Ozmen Meral Omeroglu Rukiye Eker |
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Affiliation: | Department of Pediatric Cardiology, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey. yakupergul77@hotmail.com |
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Abstract: | Aim We evaluated ambulatory patients with Duchenne muscular dystrophy from the cardiovascular standpoint and studied the correlation between the results of electrocardiographic (ECG) findings, left ventricular ejection fraction (LVEF), troponin T and N‐terminal pro‐brain natriuretic peptide (NT‐proBNP) levels and patients' North Star Ambulatory Assessment scores. Methods: Fifty patients of ages 6–12 (8.9 ± 2.8) were enrolled in this cross‐sectional study. Cardiac evaluation included electrocardiography, echocardiography and cardiac enzyme tests. Results North Star scores ranged from 6/34 to 34/34. Twenty‐eight patients (56%) had ECG changes. The most frequently seen ECG abnormalities were short PR interval (14%, n= 7), right ventricular hypertrophy (16%, n= 8), prolonged QTc interval (10%, n= 5), prominent Q wave (10%, n= 5) and T wave inversion (44%, n= 22). In 10 patients (20%), LVEF was below 55%, troponin T and NT‐proBNP levels were significantly elevated (P= 0.003 and P < 0.001, respectively). When North Star scores were compared to patients' age, enzyme levels, ECG and echocardiographic results, we discovered negative correlation with age (P < 0.001) and troponin T levels (P= 0.02) and positive correlation with LVEF (P= 0.02). Conclusion Patients with North Star scores of ≤16 are more at risk of developing cardiomyopathies. Troponin T is a cardiac index that can be used for evaluating myopathic patients and it seems to be correlated with the proBNP levels and LVEF values. |
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Keywords: | Duchenne muscular dystrophy children North Star |
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