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Unexpected perinatal death and sudden infant death syndrome (SIDS): anatomopathologic and legal aspects.

This work intends to be a review of the recent histopathological findings elicited by research into sudden and unexpected perinatal death and sudden infant death syndrome (SIDS) that have dictated a novel approach to the inherent problems by pathologists, especially those entrusted with forensic medical authority. The new approach stems from the recent advances made in the understanding of neuro- and/or cardiac-conduction-system diseases present in unexpected perinatal death and SIDS. These demand that an accurate morphologic examination be performed of these structures, which modulate respiratory, cardiovascular, digestive, and arousal activities, in all victims of sudden death. A histopathologic study of an ample register of cases of victims of sudden death, either perinatally or in early infancy, has demonstrated frequent alterations both of the autonomic nervous system (especially hypoplasia of the arcuate nucleus) and of the cardiac conduction system (accessory atrioventricular pathways). The present research provides an in-depth study of the many still-controversial aspects underlying perinatal unexpected death and SIDS and is recommended for professionals working in the forensic field, whose greater insight into this problem will allow more complete medicolegal documentation.

Autonomic Nervous System↗

Bradycardia-associated torsade de pointes and the long-QT syndromes: a case report and review of the literature.

We report a case of bradycardia-associated torsade de pointes in which the underlying long-QT syndrome appeared to be attributable to primary cardiac conducting system disease. Our patient presented complaining of presyncope and syncope. Serial electrocardiograms obtained over a period of 10 years demonstrated slowly progressive conduction system abnormalities, and evaluation revealed no other cause. The patient's dysrhythmia was refractory to magnesium but abated with cardiac pacing at a moderate rate. A review of the relevant literature on congenital and acquired long-QT syndrome is included.

Aged↗

Progressive cardiac conduction defect and cardiomyopathy in siblings with syncope.

Three siblings evaluated for syncope were found to have left bundle-branch block. Progression to complete heart block occurred in all. Pacemaker implantation eliminated syncopal epidoses in each case. Echocardiographic manifestations of cardiomyopathy were present in each child despite normal roentgenographic heart size. Careful family study identified no other affected members with conduction defects. These children area believed to represent a form of familial cardiomyopathy in which clinical manifestations of cardiac conduction system disease predominate.

Bundle-Branch Block↗

Perspective: cardiovascular disease in the postgenomic era--lessons learned and challenges ahead.

Despite remarkable advances in medical therapeutics and technology over the last 40 yr, cardiovascular disease remains the leading cause of mortality in the United States. Elucidation of the human genome and the application of gene mapping techniques to kindreds harboring rare monogenic cardiovascular syndromes have provided fundamental insights into the pathogenesis of common cardiovascular diseases including hypertension, hypercholesterolemia, cardiomyopathy with and without conduction system disease, cardiac arrhythmias, and most recently congenital heart disease. These findings led to the unanticipated conclusion that common cardiovascular pathologies (e.g. cardiomyopathy, congenital heart disease, hypertension, cardiac arrhythmias) are united by association with distinct subsets of genes. In this review, the impact of these data on the molecular pathogenesis and development of future therapies for cardiomyopathy, congenital heart disease, and atherosclerosis are highlighted. In addition, the application and limitations of evolving genetic and genomic technologies to acquired and/or multigenic cardiovascular states including atherosclerosis and high density lipoprotein (HDL) metabolism is discussed.

Animals↗

[Ageing changes of the cardiac conduct system].

This paper reviews the ageing changes of the human cardiac conduction system according to the ever published literature. In ageing process, the cardiac conduction system shows typical physiological changes: (1) differentiation, development and maturation in fetal and postnatal period, (2) reduction of the specific conduction cells and the changes of the whole node volumes. (3) fibrosis and fat infiltration. It is stressed that while explaining the causes of sudden death with the knowledge of cardiac conduction system diseases, the forensic pathologist should be able to distinguish the pathological changes from that of the normal ageing.

Age Factors↗