Little is known regarding cardiac involvement (CI) by neuromuscular disorders (NMDs). The purpose of this review is to summarise and discuss the major findings concerning the types, frequency, and severity of cardiac disorders in NMDs as well as their diagnosis, treatment, and overall outcome. CI in NMDs is characterized by pathologic involvement of the myocardium or cardiac conduction system. ...

We report a critical role for the T-box transcription factor Tbx5 in development and maturation of the cardiac conduction system. We find that Tbx5 is expressed throughout the central conduction system, including the atrioventricular bundle and bundle branch conduction system. Tbx5 haploinsufficiency in mice (Tbx5(del/+)), a model of human Holt-Oram syndrome, caused distinct morphological and f...

OBJECTIVE Noninvasive cardiac assessment of newborns and infants of women with systemic lupus erythematosus. The children had no congenital total atrioventricular block and were compared with the children of healthy women. METHODS We prospectively assessed 13 newborns and infants aged 1 to 60 days, children of women with systemic lupus erythematosus and without congenital total atrioventricul...

Correlation of electrocardiographic abnormalities occurring postoperatively with lesions of the conduction system found at autopsy was done in 20 dogs which had received cardiac homografts. The incidence of arrhythmias closely paralleled the severity of rejection injury found within the heart and specifically within the conduction system. Severe pathological changes in the conduction system (gr...

Correlation of electrocardiographic abnormalities occurring postoperatively with lesions of the conduction system found at autopsy was done in 20 dogs which had received cardiac homografts. The incidence of arrhythmias closely paralleled the severity of rejection injury found within the heart and specifically within the conduction system. Severe pathological changes in the conduction system (gr...

Retrograde conduction from ventricle to atrium is a well-recognized occurrence in the human heart. The phenomenon has been observed repeatedly during ventricular tachycardia (Kistin, 1961), with ventricular premature and interpolated beats (Kistin, 1959, 1963; Kistin and Landowne, 1951), in patients with ventricular pacemakers (Kastor and De Sanctis, 1967; Barold, Linhart, and Samet, 1968), and...

Mammalian species express nine functional voltage-gated Na(+) channels. Three of them, the cardiac-specific isoform Nav1.5 and the neuronal isoforms Nav1.8 and Nav1.9, are relatively resistant to the neurotoxin tetrodotoxin (TTX; IC50 ≥ 1 μM). The other six isoforms are highly sensitive to TTX with IC50 values in the nanomolar range. These isoforms are expressed in the central nervous system (N...