[Heart sounds and heart murmurs in progressive muscular dystrophy of Duchenne type (author's transl)].

Phonocardiographic and echocardiographic investigation was performed in patients with progressive muscular dystrophy of Duchenne type (PMD). The clinical materials consisted of 90 patients with PMD (aged 8 to 21 yrs, a mean of 14.5), and 90 normal subjects (aged 6 to 19 yrs, a mean of 11.7). The patients with PMD were classified into 8 stages from the mildest, S(1), to the severest, S(8), according to Swinyard-Deaver' criteria. In the 90 normal subjects the diminished first heart sound was noted in 12 cases (13.3%), presystolic murmurs in 4 cases (4.4%), and diastolic rumbles in 9 cases (10%), whereas, in the patients with PMD the diminished first heart sound was noted in 47 cases (52.2%), presystolic murmurs in 41 cases (45.6%), and diastolic rumbles in 44 cases (48.9%). There was a significant difference in the incidence of the above-mentioned three phonocardiographic findings between the PMD patients and the normal subjects. But there was no significant difference in the incidence of a systolic click between these two groups. The incidence of the diminished first heart sound increased with the progress of Swinyard-Deaver' classification. A presystolic murmur was observed with the highest incidence in the stage of S(8). The incidence of a rumble was also augmented with increasing severity of the disorder from the stages of S(1) to S(7), but decreased in S(8). Another attenpt was made to relate the phonocardiographic findings to those of the echocardiogram. In the cases with anterior mitral leaflet fluttering, there were diastolic rumble in 69% whereas 16.7% of the patients without anterior mitral leaflet fluttering had diastolic rumbles. In two-dimensional echocardiography, the anterior and posterior mitral leaflets looked like pennants fluttering in the wind. All these observations positively indicate that anterior mitral leaflet fluttering was closely associated with the genesis of rumbles. Consequently, it can be concluded that the diminished first heart sound, presystolic murmurs and diastolic rumbles might be useful clinical signs in the assessment of the myocardial involvement in PMD.