HF-1 b, an SP1 -related transcription factor, is preferentially expressed in the cardiac conduction system and ventricular myocytes in the heart. Mice deficient for HF-1 b survive to term and exhibit normal cardiac structure and function but display sudden cardiac death and a complete penetrance of conduction system defects, including spontaneous ventricular tachycardia and a high incidence of ...

RATIONALE The ventricular conduction system controls the propagation of electric activity through the heart to coordinate cardiac contraction. This system is composed of specialized cardiomyocytes organized in defined structures including central components and a peripheral Purkinje fiber network. How the mammalian ventricular conduction system is established during development remains controve...

RATIONALE To date, there has been no specific marker of the first heart field to facilitate understanding of contributions of the first heart field to cardiac lineages. Cardiac arrhythmia is a leading cause of death, often resulting from abnormalities in the cardiac conduction system (CCS). Understanding origins and identifying markers of CCS lineages are essential steps toward modeling disease...

A 4-month-old female infant considered to be in good health died suddenly and unexpectedly. Post- mortem examination was requested, with clinical diagnosis of sudden infant death syndrome. At autopsy the infant was described in good health. Histo- logical examination of the heart found a cardiac fibroma compressing the atrio-ventricular node and the examination of the cardiac conduction system ...

RATIONALE The gap junctional protein connexin (Cx) 45 is strongly expressed in the early embryonic myocardium. In the adult hearts of mice and humans, the expression mainly is restricted to the cardiac conduction system. Cx45 plays an essential role for development and function of the embryonic heart because general and cardiomyocyte-directed deficiencies of Cx45 in mice lead to embryonic letha...

to AVB and myocarditis, respectively, resulting in a poor prognosis. We previously reported that fetal hydrops is associated with a poor prognosis in fetuses without CHD7 and we suggested that myocardial dysfunction, rather than the severity of bradyarrhythmia, was associated with fetal hydrops. In contrast, in fetuses with CHD, congenital bradyarrhythmia is thought to result from the structura...

tricular canal. J Thorac Cardiovasc Surg 55: 299, 1968 16. McMullan MH, Wallace RB, Weidman WH: Surgical treatment of complete atrioventricular canal. Surgery 72: 905, 1972 17. Lev M: The architecture of the conduction system in congenital heart disease. 1. Common atrioventricular orifice. Arch Pathol 65: 174, 1958 18. Feldt RH. DuShane JW, Titus JL: The atrioventricular conduction system in pe...

BACKGROUND Fahr's disease is a rare neurodegenerative disorder of unknown cause characterized by idiopathic basal ganglia calcification that is associated with neuropsychiatric and cognitive impairment. No case of Fahr's disease with associated cardiac conduction disease has been described in the literature to date. The objective of this case report was to describe a young female with various c...

15. Pick A: Abberant ventricular conduction of escaped beats. Preferential and accessory pathways in the A-V junction. Circulation 13: 702, 1956 16. Kistin AD: Atrio-ventricular junctional premature and escaped beats with altered QRS and fusion. Circulation 34: 740, 1966 17. Anderson GJ, Greenspan K, Bandura JP, Fisch C: Asynchrony of conduction within the canine specialized Purkinje fiber syst...