[Comparative echocardiographic and vectorcardiographic study of 17 patients with Duchenne muscular dystrophy (author's transl)]

Abstract

Cardiac impairment in Duchenne muscular dystrophy (DMD) is well known since many years. Degeneration of myocardial fibers and progressive scarring of the left ventricle, especially in the postero-basal and lateral portions, represent the habitual cardiac pathologic features of post-mortem investigations. Nevertheless many questions about etiopathogenesis and pathophysiology of "DMD cardiomyopathy" are still debated. Clinical, vectorcardiographic (VCG) and echocardiographic (ECO) data of 17 patients suffering from DMD are here reported. Patients were subdivided in two groups according to age: A) subjects from 4 to 10 yrs. (mean 0.22 +/- 1.82 yrs.), 10 cases; B) subjects from 11 to 20 yrs. (mean 15.2 +/- 2.8 yrs.), 7 cases. Both patients groups were compared with age-matched normal controls (group A1 and B1). Our results show: 1) cardiac clinical symptoms and signs, even if is present in infancy, become more evident in adult age; 2) echocardiogram allows an early diagnosis and accurate follow-up of such cardiac pathology. Group A patients, in comparison with his own control group, exhibited a significant impairment of the left ventricular function indexes (PWE, IVSE, SV, Vcf, EF%, delta S%). Moreover the older group of patients (group B), besides the alteration of the above mentioned indexes, exhibited a significant decrease of the PWT and an impairment of DEVM. This, in agreement with other Authors, gives evidence to the progressive deteriorating of cardiac function in DMD. 3) A significant correlation between ECO and VCG data is lacking. Nevertheless VCG also displays a clear tendency to get worse with age. Vectorcardiographic features well agree with the post-mortem findings of a progressive but scattered myocardial fibrosis with elective localization in postero-basal and lateral (free wall) portions of left ventricle. 4) For the most part, in our patients, cardiological instrumental findings (ECO and VCG) are well in agreement with clinical data and natural history of "DMD cardiomyopathy". The afore said methods of investigation appear very useful in the diagnostic and therapeutic management of such patients.