뒤시엔느형 근디스트로피 환자의 심장기능 평가

Abstract

Objective: To evaluate the cardiac function and to explore the importance of the evaluation of cardiac function in patients with Duchenne muscular dystrophy (DMD). Method: Thirty-nine patients with DMD without any symptoms of heart problems underwent physical examinations and cardiac monitoring including the arterial carbon dioxide (CO2) screening. Thirty one patients underwent pulmonary function test. Results: Among 39 patients 27 showed abnormal electrocardiographic findings such as ventricular hypertrophy, ischemic change, atrial hypertrophy, T wave inversion, sinus tachycardia and ST elevation. 24 patients showed abnormal echocardiographic findings such as abnormal ejection fraction, dilated cardiomyopathy (DCMP), filling abnormality of left ventricle, global hypokinesia and reduced systolic function. 17 patients showed low ejection fraction (below 59%) and 4 of them were diagnosed as DCMP. There were significant correlations between age and ejection fraction (r=⁣0.552, p＜0.01), between functional level and ejection fraction (r=⁣0.607, p＜0.01) and between vital capacity and ejection fraction (r=0.547, p＜0.01). However, ejection fraction showed no significant correlations with arterial CO2. Conclusion: Routine evaluation of the cardiac function, at least from 10 years of age, and proper treatment following early diagnosis of heart problems were necessary in patients with DMD, because they possibly have been severely affected by cardiac problems without any clinical symptoms.